Pentalogy of Fallot: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{SI}} | {{SI}} | ||
{{CMG}} {{AE}}[[User:Abdulkareem Opeoluwalukan| | {{CMG}} {{AE}}[[User:Abdulkareem Opeoluwalukan|Abdul-Kareem Lukan, M.D]]. | ||
==Overview== | ==Overview== | ||
Pentalogy of Fallot is defined as the occurrence of Tetralogy of Fallot plus an [[atrial septal defect]].[[File:Pentalogy_of_fallot_image_1.png |200p|thumb|center| Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Private Bag X04, Onderstepoort 0110, South Africa. paolo.pazzi@up.ac.za.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3984729/]] | Pentalogy of Fallot is defined as the occurrence of Tetralogy of Fallot plus an [[atrial septal defect]].[[File:Pentalogy_of_fallot_image_1.png |200p|thumb|center| Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Private Bag X04, Onderstepoort 0110, South Africa. paolo.pazzi@up.ac.za.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3984729/]] | ||
==Historical Perspective== | ==Historical Perspective== | ||
*[[Tetrallogy of fallot|Tetralogy of fallot]] was first discovered by Niel Stenson, a Denish physician [[Anatomist]], in 1671. <ref name="pmid30020660">{{cite journal |vauthors=Diaz-Frias J, Guillaume M |title= |journal= |volume= |issue= |pages= |date= |pmid=30020660 |doi= |url=}}</ref> | |||
*[[Tetrallogy of fallot|Tetralogy of fallot]] was first discovered by Niel Stenson, a Denish physician Anatomist, in 1671. <ref name="pmid30020660">{{cite journal |vauthors=Diaz-Frias J, Guillaume M |title= |journal= |volume= |issue= |pages= |date= |pmid=30020660 |doi= |url=}}</ref> | * The association between [[right ventricular outflow tract obstruction]] and a large [[ventricular septal defect]] and [[Tetralogy of Fallot|tetralogy of fallot]] was made in 1888 by Etinne-Louise.<ref name="ApitzWebb2009">{{cite journal|last1=Apitz|first1=Christian|last2=Webb|first2=Gary D|last3=Redington|first3=Andrew N|title=Tetralogy of Fallot|journal=The Lancet|volume=374|issue=9699|year=2009|pages=1462–1471|issn=01406736|doi=10.1016/S0140-6736(09)60657-7}}</ref> | ||
* In 1924, Canadian, Maude Abbott was the first to discover the association between [[ventricular septal defect]] , [[Pulmonary valve stenosis|pulmonary stenosis]], [[overriding aorta]] and [[right ventricular hypertrophy]] and the development of [[tetralogy of Fallot]].<ref name="ApitzWebb2009">{{cite journal|last1=Apitz|first1=Christian|last2=Webb|first2=Gary D|last3=Redington|first3=Andrew N|title=Tetralogy of Fallot|journal=The Lancet|volume=374|issue=9699|year=2009|pages=1462–1471|issn=01406736|doi=10.1016/S0140-6736(09)60657-7}}</ref | |||
* The association between right ventricular outflow tract obstruction and a large [[ventricular septal defect]] and tetralogy of fallot | In 1939, the first surgical treatment was developed by Dr Helen Taussig in Johns Hopkins&amp;lt;nowiki&amp;gt;&&amp;lt;/nowiki&amp;gt;lt;nowiki&lt;nowiki&gt;&&lt;/nowiki&gt;gt;<nowiki>&</nowiki>lt;nowiki<nowiki>&</nowiki>gt;<nowiki><nowiki><ref name="pmid30020660"></nowiki></nowiki></nowiki></nowiki>{{cite journal |vauthors=Diaz-Frias J, Guillaume M |title= |journal= |volume= |issue= |pages= |date= |pmid=30020660 |doi= |url=}}</ref> | ||
* In 1924, Canadian, Maude Abbott was the first to discover the association between ventricular septal defect , pulmonary stenosis, overriding aorta and right ventricular hypertrophy and the development of tetralogy of Fallot.<ref name="ApitzWebb2009">{{cite journal|last1=Apitz|first1=Christian|last2=Webb|first2=Gary D|last3=Redington|first3=Andrew N|title=Tetralogy of Fallot|journal=The Lancet|volume=374|issue=9699|year=2009|pages=1462–1471|issn=01406736|doi=10.1016/S0140-6736(09)60657-7}}</ref | |||
In 1939, the first surgical treatment was developed by Dr Helen Taussig in Johns Hopkins&lt;nowiki&gt;&&lt;/nowiki&gt;lt;nowiki<nowiki>&</nowiki>gt; | |||
==Classification== | ==Classification== | ||
* There is no established system for the [[classification]] of the pentalogy of Fallot. | |||
* There is no established system for the classification of pentalogy of | |||
==Pathophysiology== | ==Pathophysiology== | ||
It is thought that conotruncal diseases like [[Tetralogy of Fallot|tetralogy of fallot]] and pentalogy of Fallot occur as a result of:<ref name="OMJA2020">{{cite journal|last1=OM|first1=Akinlade|last2=JA|first2=Ogunmodede|last3=OA|first3=Adeyemi|last4=WO|first4=Yusuf|last5=RO|first5=Awodun|last6=YA|first6=Ayoola|last7=IA|first7=Yusuf|last8=PM|first8=Kolo|title=Adult Nigerian with Untreated Pentalogy of Fallot: A Case Report|journal=Asploro Journal of Biomedical and Clinical Case Reports|volume=3|issue=1|year=2020|pages=62–66|issn=2582-0370|doi=10.36502/2020/ASJBCCR.6187}}</ref> | |||
It is thought that conotruncal | * [[Neural crest]] migration defect. | ||
* Underdevelopment of right ventricular infundibulum | |||
==Causes== | ==Causes== | ||
*The [[cause]] of the pentalogy of Fallot has not been identified. | |||
*To review [[risk factors]] for the development of pentalogy of Fallot, click [[Pentalogy of Fallot#Risk factors|here]]. | |||
==Differentiating pentalogy of Fallot from other Diseases== | |||
*Pentalogy of Fallot must be differentiated from other [[diseases]] that cause:<ref name="pmid30020660">{{cite journal |vauthors=Diaz-Frias J, Guillaume M |title= |journal= |volume= |issue= |pages= |date= |pmid=30020660 |doi= |url=}}</ref> | |||
The cause of pentalogy of | ===Right to left shunt=== | ||
*[[Transposition of the great vessels|Transposition of great arteries]] with [[Right ventricular outflow tract obstruction|Pulmonic stenosis]] | |||
==Differentiating pentalogy of | *[[Double outlet right ventricle]] including [[Taussig-Bing syndrome|Taussig-Bing anomaly]] | ||
*[[Tricuspid atresia]] | |||
* [[Ebstein Anomaly]] | |||
=== | *[[Pulmonary atresia]] with intact [[Interventricular septum|ventricular septum]] | ||
*[[Transposition of the great vessels| | *[[Respiratory]] diseases | ||
* | ** [[Bronchiolitis]] | ||
*[[ | ** Viral and bacterial [[Pneumonia]] | ||
* Ebstein Anomaly | ** [[Pneumothorax]] because when they are in [[Congestive heart failure|heart failure]] they present with signs of respiratory distress, [[cyanosis]] and [[failure to thrive]]. | ||
*[[ | |||
*Respiratory diseases | |||
* Bronchiolitis | |||
* | |||
*[[ | |||
=== Bidirectional shunt diseases=== | === Bidirectional shunt diseases=== | ||
* | * [[Total anomalous pulmonary venous return]] | ||
*[[ | *[[Hypoplastic left heart syndrome]] | ||
* | *[[Single ventricle]] | ||
*[[Persistent truncus arteriosus| | *[[Persistent truncus arteriosus|Truncus arteriosus]] | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The epidemiology of pentalogy of | The epidemiology of the pentalogy of Fallot has not been well studied. | ||
==Risk Factors== | ==Risk Factors== | ||
Common [[risk factors]] in the development of pentalogy of fallot include:<ref name="SilvaNeves2019">{{cite journal|last1=Silva|first1=Joana A.|last2=Neves|first2=Ana L.|last3=Flor-de-Lima|first3=Filipa|last4=Soares|first4=Paulo|last5=Guimarães|first5=Hercília|title=Risk Factors and Outcomes of Tetralogy of Fallot: From Fetal to Neonatal Life|journal=Pediatric Cardiology|volume=41|issue=1|year=2019|pages=155–164|issn=0172-0643|doi=10.1007/s00246-019-02239-4}}</ref> | |||
*[[Alcoholism]] in mother | |||
* Maternal [[Diabetes]] | |||
Common risk factors in the development of pentalogy of fallot | |||
* Alcoholism in mother | |||
* Maternal Diabetes | |||
* Advanced maternal age i.e greater than 40 years | * Advanced maternal age i.e greater than 40 years | ||
* Malnutrition in pregnancy | *[[Malnutrition]] in pregnancy | ||
* Family history of tetralogy of fallot | * Family history of [[Tetralogy of Fallot|tetralogy of fallot]] | ||
* Viral illness during pregnancy like [[Rubella]]. | * [[Viral]] illness during pregnancy like [[Rubella]]. | ||
* | * Presence of [[Down syndrome]] or [[22q11.2 deletion syndrome|DiGeorge syndrome]] | ||
==Screening== | ==Screening== | ||
There is insufficient evidence to recommend routine screening for pentalogy of | There is insufficient evidence to recommend routine screening for the pentalogy of Fallot. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
If left untreated, patients with pentalogy of fallot may progress to develop finger clubbing, stunted growth, | If left untreated, patients with pentalogy of fallot may progress to develop [[Clubbing|finger clubbing]], [[stunted growth]], [[Cardiac arrhythmia|cardiac arrhythmias]], hypoplastic pulmonary artery, [[Paradoxical embolism|paradoxical emboli]] and [[polycythemia]] .<ref name="OMJA2020">{{cite journal|last1=OM|first1=Akinlade|last2=JA|first2=Ogunmodede|last3=OA|first3=Adeyemi|last4=WO|first4=Yusuf|last5=RO|first5=Awodun|last6=YA|first6=Ayoola|last7=IA|first7=Yusuf|last8=PM|first8=Kolo|title=Adult Nigerian with Untreated Pentalogy of Fallot: A Case Report|journal=Asploro Journal of Biomedical and Clinical Case Reports|volume=3|issue=1|year=2020|pages=62–66|issn=2582-0370|doi=10.36502/2020/ASJBCCR.6187}}</ref> | ||
Prognosis is generally excellent provided the [[Ventricular septal defect|VSD]] has been closed and the [[right ventricular outflow tract]] has been relieved.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. 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Prognosis is generally excellent provided the [[Ventricular septal defect|VSD]] has been closed and the right ventricular outflow tract has been relieved.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | |||
==Diagnosis== | ==Diagnosis== | ||
===Diagnostic Study of Choice=== | ===Diagnostic Study of Choice=== | ||
There are no established criteria for the diagnosis of pentalogy Fallot. | |||
There are no established criteria for the diagnosis of pentalogy | |||
===History and Symptoms=== | ===History and Symptoms=== | ||
The hallmark of pentalogy of fallot is [[cyanosis]]. A positive history of [[cyanosis]] and easy fatigability leading to squatting during exercise is suggestive of cyanotic [[congenital]] heart defect.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | |||
The hallmark of pentalogy of fallot is cyanosis. A positive history of cyanosis and easy fatigability leading to squatting during exercise is suggestive of cyanotic congenital heart defect.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | . The most common symptoms of pentalogy of fallot include [[cyanosis]], easy fatigabillity , and hypercyanotic spells with crying , feeding and defecating.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
. The most common symptoms of pentalogy of fallot include cyanosis, easy fatigabillity , and hypercyanotic spells with crying , feeding and defecating.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | |||
==Physical Examination== | ==Physical Examination== | ||
[[Patients]] with pentalogy of Fallot appear [[cyanotic]].<ref name="OMJA2020">{{cite journal|last1=OM|first1=Akinlade|last2=JA|first2=Ogunmodede|last3=OA|first3=Adeyemi|last4=WO|first4=Yusuf|last5=RO|first5=Awodun|last6=YA|first6=Ayoola|last7=IA|first7=Yusuf|last8=PM|first8=Kolo|title=Adult Nigerian with Untreated Pentalogy of Fallot: A Case Report|journal=Asploro Journal of Biomedical and Clinical Case Reports|volume=3|issue=1|year=2020|pages=62–66|issn=2582-0370|doi=10.36502/2020/ASJBCCR.6187}}</ref><ref name="ParwaniPhillips2016">{{cite journal|last1=Parwani|first1=Purvi|last2=Phillips|first2=Sabrina|title=IMPORTANCE OF PHYSICAL EXAMINATION IN ADULT PATIENTS WITH TETRALOGY OF FALLOT|journal=Journal of the American College of Cardiology|volume=67|issue=13|year=2016|pages=1101|issn=07351097|doi=10.1016/S0735-1097(16)31102-0}}</ref> | |||
=== | * Physical examination of patients with Pentalogy of Fallot is usually remarkable for: | ||
** Right ventricular heave | |||
** Elevated jugular venous pressure | |||
** [[Clubbing|Finger clubbing]] | |||
=== | ** Displaced apex beat | ||
** Normal S1 with single S2 | |||
** [[Pedal edema|Peripheral edema]] | |||
** [[Heart murmur|Diastolic murmur]] at the left sternal border | |||
** [[Systolic murmur]] increasing with [[inspiration]] at the [[left sternal border]] | |||
=== | |||
=== | |||
* | |||
* | |||
* | |||
* | |||
* | |||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Some patients with pentalogy of fallot may have [[polycythemia]] | Some patients with pentalogy of fallot may have [[polycythemia]] | ||
which is usually suggestive of worsening hypoxia and compensatory production of erythropoetin.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | which is usually suggestive of worsening [[hypoxia]] and compensatory production of erythropoetin.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
===Electrocardiogram=== | ===Electrocardiogram=== | ||
An ECG may be helpful in the diagnosis of pentalogy of Fallot, | |||
An ECG may be helpful in the diagnosis of pentalogy of fallot <ref>{{cite journal|doi=10.1007/978-3-540-29676-8_1393 | Findings on an ECG suggestive of pentalogy of fallot include [[right axis deviation]] and [[right ventricular hypertrophy]].<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
[[File: Right_ventrriular_hypertophy.png|200p|thumb|right|Echocardiography Research Center, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3874376/]] | [[File: Right_ventrriular_hypertophy.png|200p|thumb|right|Echocardiography Research Center, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3874376/]] | ||
===X-ray=== | ===X-ray=== | ||
An x-ray may be helpful in the diagnosis of pentalogy of Fallot. Findings on an x-ray suggestive of pentalogy of fallot include boot shaped heart or coeur en sabot.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> [[File:Boot_shaped_heart.png|200p|thumb|left| Typical preoperative chest X‐ray of a 16‐month‐old boy with tetralogy of Fallot.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5412831/]] | |||
An x-ray may be helpful in the diagnosis of pentalogy of Fallot. Findings on an x-ray suggestive of pentalogy of fallot include boot shaped heart or coeur en sabot.<ref>{{cite journal|doi=10.1007/978-3-540-29676-8_1393 | |||
===Echocardiography or Ultrasound=== | ===Echocardiography or Ultrasound=== | ||
Echocardiography may be helpful in the diagnosis of pentalogy of fallot. Findings on an echocardiography diagnostic of pentalogy of fallot include.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | |||
*[[Ventricular septal defect]]. | |||
* Atrial septal defect. | |||
*[[Right ventricular outflow tract obstruction]]. | |||
Echocardiography may be helpful in the diagnosis of pentalogy of fallot. Findings on an echocardiography diagnostic of | *[[overriding aorta]] [[File:Pentalogy_of_fallot_image_2.png|200p|thumb|left | Department of Biochemistry, University of Allahabad, Allahabad-211002, India.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3299189/]] | ||
===CT scan=== | ===CT scan=== | ||
Thoracic [[CT angiography]] may be helpful in the diagnosis of pentalogy of fallot. Findings on CT scan diagnostic of pentalogy of fallot include. <ref name="LapierreDubois2016">{{cite journal|last1=Lapierre|first1=C.|last2=Dubois|first2=J.|last3=Rypens|first3=F.|last4=Raboisson|first4=M.-J.|last5=Déry|first5=J.|title=Tetralogy of Fallot: Preoperative assessment with MR and CT imaging|journal=Diagnostic and Interventional Imaging|volume=97|issue=5|year=2016|pages=531–541|issn=22115684|doi=10.1016/j.diii.2016.01.009}}</ref> | |||
*[[ventricular septal defect]]. | |||
Thoracic CT angiography may be helpful in the diagnosis of | *[[right ventricular hypertrophy]], | ||
*[[pulmonary atresia]] | |||
*[[Aortopulmonary Window|aortopulmonary]] collateral arteries. | |||
===MRI=== | ===MRI=== | ||
[[Cardiovascular magnetic resonance imaging (CMR)|cardiac MRI]] may be helpful in the diagnosis of pentalogy of fallot. Findings on MRI diagnostic of pentalogy of fallot include RVOT stenosis, [[VSD,]] [[ASD,]][[Overriding aorta]], [[right ventricular hypertrophy]] and hypoplastic pulmonary arteries.<ref name="LapierreDubois2016">{{cite journal|last1=Lapierre|first1=C.|last2=Dubois|first2=J.|last3=Rypens|first3=F.|last4=Raboisson|first4=M.-J.|last5=Déry|first5=J.|title=Tetralogy of Fallot: Preoperative assessment with MR and CT imaging|journal=Diagnostic and Interventional Imaging|volume=97|issue=5|year=2016|pages=531–541|issn=22115684|doi=10.1016/j.diii.2016.01.009}}</ref> | |||
cardiac MRI may be helpful in the diagnosis of pentalogy of fallot. Findings on MRI diagnostic of pentalogy of fallot include RVOT stenosis, VSD, ASD,Overriding aorta, right ventricular hypertrophy and hypoplastic pulmonary arteries.<ref name="LapierreDubois2016">{{cite journal|last1=Lapierre|first1=C.|last2=Dubois|first2=J.|last3=Rypens|first3=F.|last4=Raboisson|first4=M.-J.|last5=Déry|first5=J.|title=Tetralogy of Fallot: Preoperative assessment with MR and CT imaging|journal=Diagnostic and Interventional Imaging|volume=97|issue=5|year=2016|pages=531–541|issn=22115684|doi=10.1016/j.diii.2016.01.009}}</ref> | |||
===Other Imaging Findings=== | ===Other Imaging Findings=== | ||
Other imaging findings for pentalogy of fallot include Right ventricular angiopraphy | Other imaging findings for pentalogy of fallot include [[Right ventricular angiopraphy]] which demonstrates infundibular and [[pulmonary artery]] anatomy. left ventricular angiography, which demonstrates. <ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
*[[Ventricular septal defect|VSD]] | |||
*[[Atrial septal defect|ASD]], | |||
*[[Overiding aorta]] | |||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
There are no other diagnostic studies associated with pentalogy of Fallot | There are no other diagnostic studies associated with pentalogy of Fallot | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
The mainstay of treatment for pentalogy of fallot is Surgery.<ref>{{cite journal|doi=10.1007/978-3-540-29676-8_1393 | The mainstay of treatment for pentalogy of fallot is Surgery.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
Pharmacologic medical therapy is recommended among patients with severe RVOTO and hypercyanotic spells<ref>{{cite journal|doi=10.1007/978-3-540-29676-8_1393 | Pharmacologic medical therapy is recommended among patients with severe [[Right ventricular outflow tract obstruction|RVOTO]] and hypercyanotic spells.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
Pharmacologic medical therapies for pentalogy of fallot include <ref>{{cite journal|doi=10.1007/978-3-540-29676-8_1393 | Pharmacologic medical therapies for pentalogy of fallot include.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. C.|last102=Cate|first102=Hugo Ten|last103=Light|first103=Richard W.|last104=Bozza|first104=Patricia T.|last105=Salluh|first105=Jorge I.|last106=Jantz|first106=Michael A.|last107=Vargas|first107=Sergio L.|last108=Tomic|first108=Rade|last109=Roman|first109=Jesse|last110=Mobeireek|first110=Abdullah F.|last111=Khalid|first111=Mohammad|last112=Torres|first112=Harrys A.|last113=Chemaly|first113=Roy F.|last114=Tomic|first114=Rade|last115=Roman|first115=Jesse|last116=Light|first116=Richard W.|last117=Dispenzieri|first117=Angela|last118=Krause|first118=Sabine|last119=Lochmüller|first119=Hanns|last120=Weihrauch|first120=Julia|last121=Krieg|first121=Thomas|last122=Kühn|first122=Wolfgang|last123=Walz|first123=Gerd|last124=Merker|first124=Georg H.|last125=Kubitz|first125=Ralf|last126=Häussinger|first126=Dieter|last127=Schnog|first127=John-John B.|last128=Gerdes|first128=Victor E. A.|last129=Musco|first129=Giovanna|last130=Stoll|first130=Guido|last131=Toyka|first131=Klaus V.|last132=Leung|first132=Alexander K. C.|last133=Leung|first133=Alexander K. C.|last134=Robson|first134=William Lane M.|last135=Böhm|first135=Markus|last136=Luger|first136=Thomas A.|last137=Pischik|first137=Elena|last138=Kauppinen|first138=Raili|last139=Frank|first139=Jorge|last140=Schwarz|first140=Thomas|last141=Thapar|first141=Manish|last142=Bonkovsky|first142=Herbert. L.|last143=Wagner|first143=Jeffrey C.|last144=Smith|first144=A. Gordon|last145=Mela|first145=Maria|last146=Thalheimer|first146=Ulrich|last147=Samonakis|first147=Dimitrios|last148=Burroughs|first148=Andrew K.|last149=Mela|first149=Maria|last150=Thalheimer|first150=Ulrich|last151=Samonakis|first151=Dimitrios|last152=Burroughs|first152=Andrew K.|last153=Shastri|first153=Yogesh M.|last154=Stein|first154=Jürgen M.|last155=Segman|first155=Ronnen H.|last156=Shalev|first156=Arieh Y.|last157=Jacob|first157=Giris|last158=Robertson|first158=David|last159=Leung|first159=Alexander K. C.|last160=Robson|first160=William Lane M.|last161=Leung|first161=Alexander K. C.|last162=Lampinen|first162=Katja|last163=Kublickiene|first163=Karolina|last164=Kaaja|first164=Risto|last165=Lenarczyk|first165=Radosław|last166=Kowalski|first166=Oskar|last167=Kalarus|first167=Zbigniew|last168=Wichterle|first168=Dan|last169=Leung|first169=Alexander K. C.|last170=Maguire|first170=Jamie|last171=Mody|first171=Istvan|last172=Janssens|first172=Katrien|last173=Vanhoenacker|first173=Filip|last174=Van Hul|first174=Wim|last175=Gdynia|first175=Hans-Jürgen|last176=Ludolph|first176=Albert C.|last177=Leonard|first177=James V.|last178=Cate|first178=Hugo Ten|last179=Geboes|first179=Karel|last180=Van Assche|first180=Gert|last181=Cate|first181=Hugo Ten|last182=Berden|first182=Jo H. M.|last183=Van Kuppevelt|first183=Toin H.|last184=Rops|first184=Angelique L.|last185=Cate|first185=Hugo Ten|last186=Cate|first186=Hugo Ten|last187=Frank|first187=Jorge|last188=Schwarz|first188=Thomas|last189=Ambros-Rudolph|first189=Christina M.|last190=Lamounier-Zepter|first190=Valéria|last191=Thomas|first191=Christie P.|last192=Thomas|first192=Christie P.|last193=Thomas|first193=Christie P.|last194=Koch|first194=Christian A.|last195=Weinstein|first195=Lee S.|last196=Silve|first196=Caroline|last197=Leung|first197=Alexander K. C.|last198=Uitto|first198=Jouni|last199=Jiang|first199=Qiujie|last200=Leung|first200=Alexander K. C.|last201=Robson|first201=William Lane M.|last202=Leung|first202=Alexander K. C.|last203=Yildiz|first203=Orhan|last204=Kayabas|first204=Uner|last205=Hegele|first205=Richard G.|last206=Tournier|first206=Jean-Nicolas|last207=Halefoğlu|first207=Ahmet Mesrur|last208=Hutson|first208=Mary Redmond|last209=Kirby|first209=Margaret L.|last210=Okada|first210=Fumito|last211=Ando|first211=Yumiko|last212=Ono|first212=Asami|last213=Mori|first213=Hiromu|last214=Moore|first214=Timothy M.|last215=Townsley|first215=Mary I.|last216=Krauth|first216=Maria-T.|last217=Pabinger|first217=Ingrid|last218=Ballmann|first218=Manfred|last219=Hegele|first219=Richard G.|last220=Trow|first220=Terence K.|last221=McLaughlin|first221=Vallerie V.|last222=Hegele|first222=Richard G.|last223=Fetouh Yassin|first223=Atteyet-Alla|last224=Uebing|first224=Anselm|last225=Gatzoulis|first225=Michael A.|last226=Gardiner|first226=Helena M.|last227=Ma|first227=Lucia K.|last228=Ma|first228=Patrick T. S.|last229=Leung|first229=Alexander K. C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | ||
* prostaglandin E1 in neonates with ductal dependent pulmonary blood flow | |||
*[[prostaglandin E1]] in neonates with ductal dependent pulmonary blood flow | |||
*[[Morphine]] and [[ketamine]] for sedation | *[[Morphine]] and [[ketamine]] for sedation | ||
*vasopressors like [[phenylephrine]] | *vasopressors like [[phenylephrine]] | ||
Line 190: | Line 118: | ||
* iron treatment to decrease frequency of spells. | * iron treatment to decrease frequency of spells. | ||
Patients with hypercyanotic spells are treated with oxygen | Patients with hypercyanotic spells are treated with, | ||
* oxygen | |||
*volume expansion | |||
* morphine sedation. | |||
===Surgery=== | ===Surgery=== | ||
Surgery is the mainstay of treatment for the pentalogy of Fallot. interventional procedures are used to relieve pulmonary obstructions and embolize collaterals. | |||
different surgical procedures can be done to treat pentalogy of fallot and it includes.<ref name="Braun-FalcoMankin2009">{{cite journal|last1=Braun-Falco|first1=Markus|last2=Mankin|first2=Henry J.|last3=Wenger|first3=Sharon L.|last4=Braun-Falco|first4=Markus|last5=Kendall|first5=Stephan DiSean|last6=Blobe|first6=Gerard C.|last7=Weber|first7=Christoph K.|last8=Lorenz|first8=Richard|last9=Adler|first9=Guido|last10=Whitcomb|first10=David C.|last11=Yadav|first11=Dhiraj|last12=Bode|first12=Johannes|last13=Wabitsch|first13=Martin|last14=Debatin|first14=Klaus-Michael|last15=Posovzsky|first15=Pamela Fischer|last16=Hassan|first16=Akmal|last17=Megahed|first17=Mosaad|last18=Lundgren|first18=Tord M.|last19=Pfister|first19=Markus|last20=Zenner|first20=Hans-Peter|last21=Berardi|first21=Rossana|last22=Pierantoni|first22=Chiara|last23=Scartozzi|first23=Mario|last24=Cascinu|first24=Stefano|last25=Hendy|first25=Geoffrey N.|last26=Schwarz|first26=Johannes|last27=Storch|first27=Alexander|last28=Schnog|first28=John-John B.|last29=Gerdes|first29=Victor E. A.|last30=Schneider|first30=Susanne A.|last31=Bhatia|first31=Kailash P.|last32=Gittenberger-de Groot|first32=Adriana C.|last33=Bökenkamp|first33=Regina|last34=DeRuiter|first34=Marco C.|last35=Leung|first35=Alexander K. C.|last36=Wong|first36=Andrew L.|last37=Lang|first37=Undine E.|last38=Leung|first38=Alexander K. C.|last39=Leung|first39=Alexander K. C.|last40=Seyhan|first40=Muammer|last41=Hofmann|first41=Silke|last42=Bruckner-Tuderman|first42=Leena|last43=Hofmann|first43=Silke|last44=Bruckner-Tuderman|first44=Leena|last45=Hofmann|first45=Silke|last46=Bruckner-Tuderman|first46=Leena|last47=Kopp|first47=Peter|last48=Forrest|first48=Douglas|last49=Misirlioğlu|first49=Emine Dibek|last50=Alpan|first50=Nursel|last51=Oette|first51=Mark|last52=Imazio|first52=Massimo|last53=Chinnaiyan|first53=Kavitha M.|last54=Mehta|first54=Laxmi S.|last55=Bhat|first55=Mushtaq A.|last56=Bhat|first56=Javeed I.|last57=Stöber|first57=Gerald|last58=Reis|first58=André|last59=Rémi|first59=Jan|last60=Noachtar|first60=Soheyl|last61=Jurkat-Rott|first61=Karin|last62=Lehmann-Horn|first62=Frank|last63=Holzhausen|first63=Marinella|last64=Vergnolle|first64=Nathalie|last65=Schillinger|first65=Martin|last66=Gdynia|first66=Hans-Jürgen|last67=Ludolph|first67=Albert C.|last68=Namdar|first68=Thomas|last69=Eisenberger|first69=Claus Ferdinand|last70=Knoefel|first70=Wolfram Trudo|last71=Ginés|first71=Pere|last72=Guevara|first72=Mónica|last73=Cuesta-Muñoz|first73=Antonio L.|last74=Eijer|first74=Henk|last75=Böhm|first75=Markus|last76=Luger|first76=Thomas A.|last77=Güttler|first77=Flemming|last78=Guldberg|first78=Per|last79=Koch|first79=Richard|last80=Moseley|first80=Kathryn|last81=Eisenhofer|first81=Graeme|last82=Pacak|first82=Karel|last83=Leonard|first83=James V.|last84=Jaeken|first84=Jaak|last85=Becker|first85=Michael A.|last86=Stephani|first86=Ulrich|last87=Frazier|first87=Kendall S.|last88=Böhm|first88=Markus|last89=Luger|first89=Thomas A.|last90=Leung|first90=Alexander K. C.|last91=Sauve|first91=Reginald S.|last92=Matejas|first92=Verena|last93=Zenker|first93=Martin|last94=Leung|first94=Alexander K. C.|last95=Leung|first95=Alexander K. C.|last96=Robson|first96=William Lane M.|last97=Beissert|first97=Stefan|last98=Leung|first98=Alexander K. C.|last99=Robson|first99=William Lane M.|last100=Woo|first100=Tom Y.|last101=Leung|first101=Alexander K. 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C.|last230=Prieto|first230=Lourdes|last231=Latson|first231=Larry|last232=Keller|first232=Nancy R.|last233=Robertson|first233=David|last234=Fairbanks|first234=Lynette D.|last235=Simmonds|first235=H. Anne|last236=Brunner|first236=Martina|last237=Zouboulis|first237=Christos C.|last238=Everts|first238=Vincent|last239=Helfrich|first239=Miep|last240=Leung|first240=Alexander K. C.|last241=Wong|first241=Andrew L.|last242=Von Den Driesch|first242=Peter|last243=Bitsch|first243=Roland|last244=Bitsch|first244=Roland|last245=Bitsch|first245=Roland|last246=García-Cazorla|first246=Angels|last247=Saudubray|first247=Jean Marie|title=Pentalogy of Fallot|year=2009|pages=1602–1605|doi=10.1007/978-3-540-29676-8_1393}}</ref> | |||
* Pulmonary valvotomy( for stenotic pulmonic valve) | |||
Surgery is the mainstay of treatment for pentalogy of | * Pulmonary valvectomy( for small and thickened pulmonic valve) | ||
different surgical procedures can be done to treat pentalogy of fallot and it includes | * Closure of [[Atrial septal defect|ASD]]/[[Ventricular septal defect|VSD]] | ||
* | * Shunt surgery for severe [[cyanosis]] or in patients with frequent spells in the first year of life | ||
* | * Blalock -Taussig Shunt- communication between the right or left [[pulmonary artery]] and the right or left [[subclavian artery]]. | ||
* | * Waterson-Cooley Shunt- communication between the [[ascending aorta]] with the right [[pulmonary artery]] | ||
* | * Potts shunt-communication between the [[descending aorta]] and left [[pulmonary artery]] | ||
* Blalock -Taussig Shunt- communication between the right or left pulmonary artery and the right or left subclavian artery. | * Central shunt- Communication between the main [[Pulmonary artery|pulmonary arter]]<nowiki/>y and [[ascending aorta.]] | ||
* Waterson-Cooley Shunt- communication between the ascending aorta with the right pulmonary artery | |||
* Potts shunt-communication between the descending aorta and left pulmonary artery | |||
* Central shunt- Communication between the main pulmonary | |||
===Primary Prevention=== | ===Primary Prevention=== | ||
There are no established measures for the primary prevention of pentalogy of | There are no established measures for the primary prevention of the pentalogy of Fallot. | ||
===Secondary Prevention=== | ===Secondary Prevention=== | ||
There are no established measures for the secondary prevention of pentalogy of | There are no established measures for the secondary prevention of the pentalogy of Fallot. | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} |
Latest revision as of 04:22, 26 July 2020
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Abdul-Kareem Lukan, M.D.
Overview
Pentalogy of Fallot is defined as the occurrence of Tetralogy of Fallot plus an atrial septal defect.
Historical Perspective
- Tetralogy of fallot was first discovered by Niel Stenson, a Denish physician Anatomist, in 1671. [1]
- The association between right ventricular outflow tract obstruction and a large ventricular septal defect and tetralogy of fallot was made in 1888 by Etinne-Louise.[2]
- In 1924, Canadian, Maude Abbott was the first to discover the association between ventricular septal defect , pulmonary stenosis, overriding aorta and right ventricular hypertrophy and the development of tetralogy of Fallot.[2]
Classification
- There is no established system for the classification of the pentalogy of Fallot.
Pathophysiology
It is thought that conotruncal diseases like tetralogy of fallot and pentalogy of Fallot occur as a result of:[3]
- Neural crest migration defect.
- Underdevelopment of right ventricular infundibulum
Causes
- The cause of the pentalogy of Fallot has not been identified.
- To review risk factors for the development of pentalogy of Fallot, click here.
Differentiating pentalogy of Fallot from other Diseases
Right to left shunt
- Transposition of great arteries with Pulmonic stenosis
- Double outlet right ventricle including Taussig-Bing anomaly
- Tricuspid atresia
- Ebstein Anomaly
- Pulmonary atresia with intact ventricular septum
- Respiratory diseases
- Bronchiolitis
- Viral and bacterial Pneumonia
- Pneumothorax because when they are in heart failure they present with signs of respiratory distress, cyanosis and failure to thrive.
Bidirectional shunt diseases
- Total anomalous pulmonary venous return
- Hypoplastic left heart syndrome
- Single ventricle
- Truncus arteriosus
Epidemiology and Demographics
The epidemiology of the pentalogy of Fallot has not been well studied.
Risk Factors
Common risk factors in the development of pentalogy of fallot include:[4]
- Alcoholism in mother
- Maternal Diabetes
- Advanced maternal age i.e greater than 40 years
- Malnutrition in pregnancy
- Family history of tetralogy of fallot
- Viral illness during pregnancy like Rubella.
- Presence of Down syndrome or DiGeorge syndrome
Screening
There is insufficient evidence to recommend routine screening for the pentalogy of Fallot.
Natural History, Complications, and Prognosis
If left untreated, patients with pentalogy of fallot may progress to develop finger clubbing, stunted growth, cardiac arrhythmias, hypoplastic pulmonary artery, paradoxical emboli and polycythemia .[3] Prognosis is generally excellent provided the VSD has been closed and the right ventricular outflow tract has been relieved.[5]
Diagnosis
Diagnostic Study of Choice
There are no established criteria for the diagnosis of pentalogy Fallot.
History and Symptoms
The hallmark of pentalogy of fallot is cyanosis. A positive history of cyanosis and easy fatigability leading to squatting during exercise is suggestive of cyanotic congenital heart defect.[5] . The most common symptoms of pentalogy of fallot include cyanosis, easy fatigabillity , and hypercyanotic spells with crying , feeding and defecating.[5]
Physical Examination
Patients with pentalogy of Fallot appear cyanotic.[3][6]
- Physical examination of patients with Pentalogy of Fallot is usually remarkable for:
- Right ventricular heave
- Elevated jugular venous pressure
- Finger clubbing
- Displaced apex beat
- Normal S1 with single S2
- Peripheral edema
- Diastolic murmur at the left sternal border
- Systolic murmur increasing with inspiration at the left sternal border
Laboratory Findings
Some patients with pentalogy of fallot may have polycythemia which is usually suggestive of worsening hypoxia and compensatory production of erythropoetin.[5]
Electrocardiogram
An ECG may be helpful in the diagnosis of pentalogy of Fallot, Findings on an ECG suggestive of pentalogy of fallot include right axis deviation and right ventricular hypertrophy.[5]
X-ray
An x-ray may be helpful in the diagnosis of pentalogy of Fallot. Findings on an x-ray suggestive of pentalogy of fallot include boot shaped heart or coeur en sabot.[5]
Echocardiography or Ultrasound
Echocardiography may be helpful in the diagnosis of pentalogy of fallot. Findings on an echocardiography diagnostic of pentalogy of fallot include.[5]
- Ventricular septal defect.
- Atrial septal defect.
- Right ventricular outflow tract obstruction.
- overriding aorta
CT scan
Thoracic CT angiography may be helpful in the diagnosis of pentalogy of fallot. Findings on CT scan diagnostic of pentalogy of fallot include. [7]
- ventricular septal defect.
- right ventricular hypertrophy,
- pulmonary atresia
- aortopulmonary collateral arteries.
MRI
cardiac MRI may be helpful in the diagnosis of pentalogy of fallot. Findings on MRI diagnostic of pentalogy of fallot include RVOT stenosis, VSD, ASD,Overriding aorta, right ventricular hypertrophy and hypoplastic pulmonary arteries.[7]
Other Imaging Findings
Other imaging findings for pentalogy of fallot include Right ventricular angiopraphy which demonstrates infundibular and pulmonary artery anatomy. left ventricular angiography, which demonstrates. [5]
Other Diagnostic Studies
There are no other diagnostic studies associated with pentalogy of Fallot
Treatment
Medical Therapy
The mainstay of treatment for pentalogy of fallot is Surgery.[5]
Pharmacologic medical therapy is recommended among patients with severe RVOTO and hypercyanotic spells.[5]
Pharmacologic medical therapies for pentalogy of fallot include.[5]
- prostaglandin E1 in neonates with ductal dependent pulmonary blood flow
- Morphine and ketamine for sedation
- vasopressors like phenylephrine
- propanolol to decrease the need for palliative surgical intervention.
- iron treatment to decrease frequency of spells.
Patients with hypercyanotic spells are treated with,
- oxygen
- volume expansion
- morphine sedation.
Surgery
Surgery is the mainstay of treatment for the pentalogy of Fallot. interventional procedures are used to relieve pulmonary obstructions and embolize collaterals. different surgical procedures can be done to treat pentalogy of fallot and it includes.[5]
- Pulmonary valvotomy( for stenotic pulmonic valve)
- Pulmonary valvectomy( for small and thickened pulmonic valve)
- Closure of ASD/VSD
- Shunt surgery for severe cyanosis or in patients with frequent spells in the first year of life
- Blalock -Taussig Shunt- communication between the right or left pulmonary artery and the right or left subclavian artery.
- Waterson-Cooley Shunt- communication between the ascending aorta with the right pulmonary artery
- Potts shunt-communication between the descending aorta and left pulmonary artery
- Central shunt- Communication between the main pulmonary artery and ascending aorta.
Primary Prevention
There are no established measures for the primary prevention of the pentalogy of Fallot.
Secondary Prevention
There are no established measures for the secondary prevention of the pentalogy of Fallot.
References
- ↑ 1.0 1.1 Diaz-Frias J, Guillaume M. PMID 30020660. Missing or empty
|title=
(help) - ↑ 2.0 2.1 Apitz, Christian; Webb, Gary D; Redington, Andrew N (2009). "Tetralogy of Fallot". The Lancet. 374 (9699): 1462–1471. doi:10.1016/S0140-6736(09)60657-7. ISSN 0140-6736.
- ↑ 3.0 3.1 3.2 OM, Akinlade; JA, Ogunmodede; OA, Adeyemi; WO, Yusuf; RO, Awodun; YA, Ayoola; IA, Yusuf; PM, Kolo (2020). "Adult Nigerian with Untreated Pentalogy of Fallot: A Case Report". Asploro Journal of Biomedical and Clinical Case Reports. 3 (1): 62–66. doi:10.36502/2020/ASJBCCR.6187. ISSN 2582-0370.
- ↑ Silva, Joana A.; Neves, Ana L.; Flor-de-Lima, Filipa; Soares, Paulo; Guimarães, Hercília (2019). "Risk Factors and Outcomes of Tetralogy of Fallot: From Fetal to Neonatal Life". Pediatric Cardiology. 41 (1): 155–164. doi:10.1007/s00246-019-02239-4. ISSN 0172-0643.
- ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 Braun-Falco, Markus; Mankin, Henry J.; Wenger, Sharon L.; Braun-Falco, Markus; Kendall, Stephan DiSean; Blobe, Gerard C.; Weber, Christoph K.; Lorenz, Richard; Adler, Guido; Whitcomb, David C.; Yadav, Dhiraj; Bode, Johannes; Wabitsch, Martin; Debatin, Klaus-Michael; Posovzsky, Pamela Fischer; Hassan, Akmal; Megahed, Mosaad; Lundgren, Tord M.; Pfister, Markus; Zenner, Hans-Peter; Berardi, Rossana; Pierantoni, Chiara; Scartozzi, Mario; Cascinu, Stefano; Hendy, Geoffrey N.; Schwarz, Johannes; Storch, Alexander; Schnog, John-John B.; Gerdes, Victor E. A.; Schneider, Susanne A.; Bhatia, Kailash P.; Gittenberger-de Groot, Adriana C.; Bökenkamp, Regina; DeRuiter, Marco C.; Leung, Alexander K. C.; Wong, Andrew L.; Lang, Undine E.; Leung, Alexander K. C.; Leung, Alexander K. C.; Seyhan, Muammer; Hofmann, Silke; Bruckner-Tuderman, Leena; Hofmann, Silke; Bruckner-Tuderman, Leena; Hofmann, Silke; Bruckner-Tuderman, Leena; Kopp, Peter; Forrest, Douglas; Misirlioğlu, Emine Dibek; Alpan, Nursel; Oette, Mark; Imazio, Massimo; Chinnaiyan, Kavitha M.; Mehta, Laxmi S.; Bhat, Mushtaq A.; Bhat, Javeed I.; Stöber, Gerald; Reis, André; Rémi, Jan; Noachtar, Soheyl; Jurkat-Rott, Karin; Lehmann-Horn, Frank; Holzhausen, Marinella; Vergnolle, Nathalie; Schillinger, Martin; Gdynia, Hans-Jürgen; Ludolph, Albert C.; Namdar, Thomas; Eisenberger, Claus Ferdinand; Knoefel, Wolfram Trudo; Ginés, Pere; Guevara, Mónica; Cuesta-Muñoz, Antonio L.; Eijer, Henk; Böhm, Markus; Luger, Thomas A.; Güttler, Flemming; Guldberg, Per; Koch, Richard; Moseley, Kathryn; Eisenhofer, Graeme; Pacak, Karel; Leonard, James V.; Jaeken, Jaak; Becker, Michael A.; Stephani, Ulrich; Frazier, Kendall S.; Böhm, Markus; Luger, Thomas A.; Leung, Alexander K. C.; Sauve, Reginald S.; Matejas, Verena; Zenker, Martin; Leung, Alexander K. C.; Leung, Alexander K. C.; Robson, William Lane M.; Beissert, Stefan; Leung, Alexander K. C.; Robson, William Lane M.; Woo, Tom Y.; Leung, Alexander K. C.; Cate, Hugo Ten; Light, Richard W.; Bozza, Patricia T.; Salluh, Jorge I.; Jantz, Michael A.; Vargas, Sergio L.; Tomic, Rade; Roman, Jesse; Mobeireek, Abdullah F.; Khalid, Mohammad; Torres, Harrys A.; Chemaly, Roy F.; Tomic, Rade; Roman, Jesse; Light, Richard W.; Dispenzieri, Angela; Krause, Sabine; Lochmüller, Hanns; Weihrauch, Julia; Krieg, Thomas; Kühn, Wolfgang; Walz, Gerd; Merker, Georg H.; Kubitz, Ralf; Häussinger, Dieter; Schnog, John-John B.; Gerdes, Victor E. A.; Musco, Giovanna; Stoll, Guido; Toyka, Klaus V.; Leung, Alexander K. C.; Leung, Alexander K. C.; Robson, William Lane M.; Böhm, Markus; Luger, Thomas A.; Pischik, Elena; Kauppinen, Raili; Frank, Jorge; Schwarz, Thomas; Thapar, Manish; Bonkovsky, Herbert. L.; Wagner, Jeffrey C.; Smith, A. Gordon; Mela, Maria; Thalheimer, Ulrich; Samonakis, Dimitrios; Burroughs, Andrew K.; Mela, Maria; Thalheimer, Ulrich; Samonakis, Dimitrios; Burroughs, Andrew K.; Shastri, Yogesh M.; Stein, Jürgen M.; Segman, Ronnen H.; Shalev, Arieh Y.; Jacob, Giris; Robertson, David; Leung, Alexander K. C.; Robson, William Lane M.; Leung, Alexander K. C.; Lampinen, Katja; Kublickiene, Karolina; Kaaja, Risto; Lenarczyk, Radosław; Kowalski, Oskar; Kalarus, Zbigniew; Wichterle, Dan; Leung, Alexander K. C.; Maguire, Jamie; Mody, Istvan; Janssens, Katrien; Vanhoenacker, Filip; Van Hul, Wim; Gdynia, Hans-Jürgen; Ludolph, Albert C.; Leonard, James V.; Cate, Hugo Ten; Geboes, Karel; Van Assche, Gert; Cate, Hugo Ten; Berden, Jo H. M.; Van Kuppevelt, Toin H.; Rops, Angelique L.; Cate, Hugo Ten; Cate, Hugo Ten; Frank, Jorge; Schwarz, Thomas; Ambros-Rudolph, Christina M.; Lamounier-Zepter, Valéria; Thomas, Christie P.; Thomas, Christie P.; Thomas, Christie P.; Koch, Christian A.; Weinstein, Lee S.; Silve, Caroline; Leung, Alexander K. C.; Uitto, Jouni; Jiang, Qiujie; Leung, Alexander K. C.; Robson, William Lane M.; Leung, Alexander K. C.; Yildiz, Orhan; Kayabas, Uner; Hegele, Richard G.; Tournier, Jean-Nicolas; Halefoğlu, Ahmet Mesrur; Hutson, Mary Redmond; Kirby, Margaret L.; Okada, Fumito; Ando, Yumiko; Ono, Asami; Mori, Hiromu; Moore, Timothy M.; Townsley, Mary I.; Krauth, Maria-T.; Pabinger, Ingrid; Ballmann, Manfred; Hegele, Richard G.; Trow, Terence K.; McLaughlin, Vallerie V.; Hegele, Richard G.; Fetouh Yassin, Atteyet-Alla; Uebing, Anselm; Gatzoulis, Michael A.; Gardiner, Helena M.; Ma, Lucia K.; Ma, Patrick T. S.; Leung, Alexander K. C.; Prieto, Lourdes; Latson, Larry; Keller, Nancy R.; Robertson, David; Fairbanks, Lynette D.; Simmonds, H. Anne; Brunner, Martina; Zouboulis, Christos C.; Everts, Vincent; Helfrich, Miep; Leung, Alexander K. C.; Wong, Andrew L.; Von Den Driesch, Peter; Bitsch, Roland; Bitsch, Roland; Bitsch, Roland; García-Cazorla, Angels; Saudubray, Jean Marie (2009). "Pentalogy of Fallot": 1602–1605. doi:10.1007/978-3-540-29676-8_1393.
- ↑ Parwani, Purvi; Phillips, Sabrina (2016). "IMPORTANCE OF PHYSICAL EXAMINATION IN ADULT PATIENTS WITH TETRALOGY OF FALLOT". Journal of the American College of Cardiology. 67 (13): 1101. doi:10.1016/S0735-1097(16)31102-0. ISSN 0735-1097.
- ↑ 7.0 7.1 Lapierre, C.; Dubois, J.; Rypens, F.; Raboisson, M.-J.; Déry, J. (2016). "Tetralogy of Fallot: Preoperative assessment with MR and CT imaging". Diagnostic and Interventional Imaging. 97 (5): 531–541. doi:10.1016/j.diii.2016.01.009. ISSN 2211-5684.