Congenital heart block: Difference between revisions

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'''For patient information page click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information page click [[{{PAGENAME}} (patient information)|here]]'''


{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}; '''Contributors:'''  [[User:Lakeadam|Adam C. Lake]]
{{CMG}}; {{AE}} {{S.G.}} {{CZ}}; '''Contributors:'''  [[User:Lakeadam|Adam C. Lake]]


==Overview==
==Overview==
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==Historical Perspective==
==Historical Perspective==
*[Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Congenital heart block was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
   
   
==Classification==
==Classification==
*Congenital Heart Block may be classified according to cause into 3 subtypes/groups:
*Congenital Heart Block may be classified according to cause into 3 groups:<ref name="BaruteauPass2016">{{cite journal|last1=Baruteau|first1=Alban-Elouen|last2=Pass|first2=Robert H.|last3=Thambo|first3=Jean-Benoit|last4=Behaghel|first4=Albin|last5=Le Pennec|first5=Solène|last6=Perdreau|first6=Elodie|last7=Combes|first7=Nicolas|last8=Liberman|first8=Leonardo|last9=McLeod|first9=Christopher J.|title=Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management|journal=European Journal of Pediatrics|volume=175|issue=9|year=2016|pages=1235–1248|issn=0340-6199|doi=10.1007/s00431-016-2748-0}}</ref>
:*Autoimmune
 
:*Structural
:*First Degree Congenital Heart Block
:*Other
:*Second Degree Congenital Heart Block
*Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
:*Third Degree Congenital (Complete) Heart Block


==Pathophysiology==
==Pathophysiology==
The normal physiology of congenital heart block can be understood as follows:
The normal physiology of congenital heart block can be understood as follows:<ref name="pmid29176145">{{cite journal |vauthors=Zhou KY, Hua YM |title=Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life |journal=Chin. Med. J. |volume=130 |issue=23 |pages=2863–2871 |date=December 2017 |pmid=29176145 |pmc=5717867 |doi=10.4103/0366-6999.219160 |url=}}</ref><ref name="pmid23410880">{{cite journal |vauthors=Fahed AC, Gelb BD, Seidman JG, Seidman CE |title=Genetics of congenital heart disease: the glass half empty |journal=Circ. Res. |volume=112 |issue=4 |pages=707–20 |date=February 2013 |pmid=23410880 |pmc=3827691 |doi=10.1161/CIRCRESAHA.112.300853 |url=}}</ref><ref name="pmid221830632">{{cite journal |vauthors=Capone C, Buyon JP, Friedman DM, Frishman WH |title=Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population |journal=Cardiol Rev |volume=20 |issue=2 |pages=72–6 |date=2012 |pmid=22183063 |pmc=3275696 |doi=10.1097/CRD.0b013e31823c808b |url=}}</ref><ref name="SharmaLinden2010">{{cite journal|last1=Sharma|first1=Gaurav|last2=Linden|first2=Michael D.|last3=Schultz|first3=Daniel S.|last4=Inamdar|first4=Kedar V.|title=Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart|journal=Cardiovascular Pathology|volume=19|issue=3|year=2010|pages=e75–e78|issn=10548807|doi=10.1016/j.carpath.2008.10.011}}</ref>


*Mother's antibodies cross from the fetus placenta and damage of cardiac conduction system.  The majority of anti bodies are from mother to response an infectionin  or in response to an autoimmune disorder (SLE).<ref name="pmid29176145">{{cite journal |vauthors=Zhou KY, Hua YM |title=Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life |journal=Chin. Med. J. |volume=130 |issue=23 |pages=2863–2871 |date=December 2017 |pmid=29176145 |pmc=5717867 |doi=10.4103/0366-6999.219160 |url=}}</ref>
*Mother's antibodies cross from the fetus placenta and damage of cardiac conduction system.  The majority of anti bodies are from mother to response an infectionin  or in response to an autoimmune disorder (SLE).


* Genetic disorde (autosomal recessive/dominant genetic trait)<ref name="pmid23410880">{{cite journal |vauthors=Fahed AC, Gelb BD, Seidman JG, Seidman CE |title=Genetics of congenital heart disease: the glass half empty |journal=Circ. Res. |volume=112 |issue=4 |pages=707–20 |date=February 2013 |pmid=23410880 |pmc=3827691 |doi=10.1161/CIRCRESAHA.112.300853 |url=}}</ref>
* Genetic disorde (autosomal recessive/dominant genetic trait).
* In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).<ref name="pmid221830632">{{cite journal |vauthors=Capone C, Buyon JP, Friedman DM, Frishman WH |title=Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population |journal=Cardiol Rev |volume=20 |issue=2 |pages=72–6 |date=2012 |pmid=22183063 |pmc=3275696 |doi=10.1097/CRD.0b013e31823c808b |url=}}</ref>
* In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).
*Fibrous tissue that either replaces the atrioventricular (AV) node and its surrounding tissue or by an interruption between the atrial myocardium and the AV nod.<ref name="SharmaLinden2010">{{cite journal|last1=Sharma|first1=Gaurav|last2=Linden|first2=Michael D.|last3=Schultz|first3=Daniel S.|last4=Inamdar|first4=Kedar V.|title=Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart|journal=Cardiovascular Pathology|volume=19|issue=3|year=2010|pages=e75–e78|issn=10548807|doi=10.1016/j.carpath.2008.10.011}}</ref>
*Fibrous tissue that either replaces the atrioventricular (AV) node and its surrounding tissue or by an interruption between the atrial myocardium and the AV nod.


==Causes==
==Causes==
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==Differentiating [disease name] from other Diseases==
==Differentiating [disease name] from other Diseases==
*[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
*Congenital heart block must be differentiated from other [[Disease|diseases]]:<ref name="LipshultzLaw2019">{{cite journal|last1=Lipshultz|first1=Steven E.|last2=Law|first2=Yuk M.|last3=Asante-Korang|first3=Alfred|last4=Austin|first4=Eric D.|last5=Dipchand|first5=Anne I.|last6=Everitt|first6=Melanie D.|last7=Hsu|first7=Daphne T.|last8=Lin|first8=Kimberly Y.|last9=Price|first9=Jack F.|last10=Wilkinson|first10=James D.|last11=Colan|first11=Steven D.|title=Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association|journal=Circulation|volume=140|issue=1|year=2019|issn=0009-7322|doi=10.1161/CIR.0000000000000682}}</ref>
:*[Differential dx1]
**[[Pediatrics|Pediatric]] [[viral myocarditis]]
:*[Differential dx2]
**[[Transposition of the great vessels|Transposition of the Great Arteries]]
:*[Differential dx3]
 
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide. <ref>{{Cite journal
* The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide. <ref>{{Cite journal
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===Gender===
===Gender===
*[Disease name] affects men and women equally.
*Congenital heart block affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
   
   
===Race===
===Race===
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==Risk Factors==
==Risk Factors==
One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as [[SLE|lupus]].<ref name="pmid223686293">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>


Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.<ref name="pmid223686293" />
* One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as [[SLE|lupus]].<ref name="pmid223686293">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>
* Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.<ref name="pmid223686293" />


==Natural History, Complications, Prognosis==
==Natural History, Complications, Prognosis==
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The damaged heart may beat extremely slowly.  In some cases, the heart rate is so slow that it is fatal in nearly 20% of affected babies (with most deaths occurring as [[ fetal demise]]s).  Patients presenting as fetuses or at birth have significantly higher morbidity and mortality rates than do patients presenting later in childhood.
The damaged heart may beat extremely slowly.  In some cases, the heart rate is so slow that it is fatal in nearly 20% of affected babies (with most deaths occurring as [[ fetal demise]]s).  Patients presenting as fetuses or at birth have significantly higher morbidity and mortality rates than do patients presenting later in childhood.


*The majority of patients with [disease name] remain asymptomatic for [duration/years].
*Early clinical features include skin rash, liver
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].


== Diagnosis ==
== Diagnosis ==
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*[[Diagnosis]] of congenital heart block is confirmed by [[maternal]] [[fetal]] monitoring (MFM) in during [[infancy]] or early [[childhood]].<ref name="DonofrioMoon-Grady2014">{{cite journal|last1=Donofrio|first1=Mary T.|last2=Moon-Grady|first2=Anita J.|last3=Hornberger|first3=Lisa K.|last4=Copel|first4=Joshua A.|last5=Sklansky|first5=Mark S.|last6=Abuhamad|first6=Alfred|last7=Cuneo|first7=Bettina F.|last8=Huhta|first8=James C.|last9=Jonas|first9=Richard A.|last10=Krishnan|first10=Anita|last11=Lacey|first11=Stephanie|last12=Lee|first12=Wesley|last13=Michelfelder|first13=Erik C.|last14=Rempel|first14=Gwen R.|last15=Silverman|first15=Norman H.|last16=Spray|first16=Thomas L.|last17=Strasburger|first17=Janette F.|last18=Tworetzky|first18=Wayne|last19=Rychik|first19=Jack|title=Diagnosis and Treatment of Fetal Cardiac Disease|journal=Circulation|volume=129|issue=21|year=2014|pages=2183–2242|issn=0009-7322|doi=10.1161/01.cir.0000437597.44550.5d}}</ref>
*[[Diagnosis]] of congenital heart block is confirmed by [[maternal]] [[fetal]] monitoring (MFM) in during [[infancy]] or early [[childhood]].<ref name="DonofrioMoon-Grady2014">{{cite journal|last1=Donofrio|first1=Mary T.|last2=Moon-Grady|first2=Anita J.|last3=Hornberger|first3=Lisa K.|last4=Copel|first4=Joshua A.|last5=Sklansky|first5=Mark S.|last6=Abuhamad|first6=Alfred|last7=Cuneo|first7=Bettina F.|last8=Huhta|first8=James C.|last9=Jonas|first9=Richard A.|last10=Krishnan|first10=Anita|last11=Lacey|first11=Stephanie|last12=Lee|first12=Wesley|last13=Michelfelder|first13=Erik C.|last14=Rempel|first14=Gwen R.|last15=Silverman|first15=Norman H.|last16=Spray|first16=Thomas L.|last17=Strasburger|first17=Janette F.|last18=Tworetzky|first18=Wayne|last19=Rychik|first19=Jack|title=Diagnosis and Treatment of Fetal Cardiac Disease|journal=Circulation|volume=129|issue=21|year=2014|pages=2183–2242|issn=0009-7322|doi=10.1161/01.cir.0000437597.44550.5d}}</ref>
*First degree:<ref name="KusumotoSchoenfeld2019">{{cite journal|last1=Kusumoto|first1=Fred M.|last2=Schoenfeld|first2=Mark H.|last3=Barrett|first3=Coletta|last4=Edgerton|first4=James R.|last5=Ellenbogen|first5=Kenneth A.|last6=Gold|first6=Michael R.|last7=Goldschlager|first7=Nora F.|last8=Hamilton|first8=Robert M.|last9=Joglar|first9=José A.|last10=Kim|first10=Robert J.|last11=Lee|first11=Richard|last12=Marine|first12=Joseph E.|last13=McLeod|first13=Christopher J.|last14=Oken|first14=Keith R.|last15=Patton|first15=Kristen K.|last16=Pellegrini|first16=Cara N.|last17=Selzman|first17=Kimberly A.|last18=Thompson|first18=Annemarie|last19=Varosy|first19=Paul D.|title=2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay|journal=Journal of the American College of Cardiology|volume=74|issue=7|year=2019|pages=e51–e156|issn=07351097|doi=10.1016/j.jacc.2018.10.044}}</ref>
**Patient [[asymptomatc]]
*Second degree:
**[[Patient|Patients]] with second degree of heart block maybe have [[Symptom|symptoms]] such as:
***[[Syncope (medicine)|Fainting]]
***Feeling [[Dizziness|dizzy]]
***Feeling [[Fatigue|tired]]
*Third degree
**[[Patients]] with third degree of congenital heart block has [[Symptom|symptoms]] same as second degree but more severe.


   
   
=== Symptoms ===
=== Symptoms ===
*[[Signs]] and [[symptoms]] depend on the type of heart block the child has. [[First-degree heart block]] rarely causes [[symptom]]<nowiki/>s.
*[[Signs]] and [[symptoms]] depend on the type of heart block the child has. [[First-degree heart block]] rarely causes [[symptom]]<nowiki/>s.<ref name="BaruteauPass20162">{{cite journal|last1=Baruteau|first1=Alban-Elouen|last2=Pass|first2=Robert H.|last3=Thambo|first3=Jean-Benoit|last4=Behaghel|first4=Albin|last5=Le Pennec|first5=Solène|last6=Perdreau|first6=Elodie|last7=Combes|first7=Nicolas|last8=Liberman|first8=Leonardo|last9=McLeod|first9=Christopher J.|title=Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management|journal=European Journal of Pediatrics|volume=175|issue=9|year=2016|pages=1235–1248|issn=0340-6199|doi=10.1007/s00431-016-2748-0}}</ref>
*[[Symptom|Symptoms]] of [[second-degree heart block|second-]] and [[third-degree heart block]] include:
*[[Symptom|Symptoms]] of [[second-degree heart block|second-]] and [[third-degree heart block]] include:
**[[Fainting]]
*First degree:<ref name="KusumotoSchoenfeld2019" />
**[[Dizziness]] or [[light-headedness]]
**[[Patient]] with first degree of congenital heart block are [[asymptomatc]].
**[[Fatigue]] ([[tiredness]])
*Second degree:
**[[Shortness of breath]]
**[[Patients]] with second degree of heart block maybe have symptoms such as:
**[[Chest pain]]
***[[Fainting]]
***Feeling [[Dizziness|dizzy]]
***Feeling [[Fatigue|tired]]
*Third degree
**[[Patients]] with third degree of congenital heart block has [[Symptom|symptoms]] same as second degree but more severe.


=== Physical Examination ===
=== Physical Examination ===
*Patients with congenital heart block usually appear [general appearance].
*[[Patient|Patients]] with congenital heart block usually appear :<ref name="pmid223686294">{{cite journal |vauthors=Friedman D, Duncanson Lj, Glickstein J, Buyon J |title=A review of congenital heart block |journal=Images Paediatr Cardiol |volume=5 |issue=3 |pages=36–48 |date=July 2003 |pmid=22368629 |pmc=3232542 |doi= |url=}}</ref>
*Physical examination may be remarkable for:
 
:*[finding 1]
:*Annular lesions (Face, particularly around eyes)
:*[finding 2]
:*Photosensitive
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]


=== Laboratory Findings ===
=== Laboratory Findings ===
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*May be [[Autoantibody|autoantibodies]] to Ro/SSA and La/SSB [[Ribonucleoprotein|ribonucleoproteins]] using ELISAW are helpful when when congenital heart block  detected.<ref name="pmid19567621">{{cite journal |vauthors=Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R, Ramoni V, Vignati G, Martinelli S, Sadou Y, Borghi A, Tincani A, Chan EK, Ruffatti A |title=Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases |journal=J. Rheumatol. |volume=36 |issue=8 |pages=1744–8 |date=August 2009 |pmid=19567621 |pmc=2798588 |doi=10.3899/jrheum.080737 |url=}}</ref>
*May be [[Autoantibody|autoantibodies]] to Ro/SSA and La/SSB [[Ribonucleoprotein|ribonucleoproteins]] using ELISAW are helpful when when congenital heart block  detected.<ref name="pmid19567621">{{cite journal |vauthors=Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R, Ramoni V, Vignati G, Martinelli S, Sadou Y, Borghi A, Tincani A, Chan EK, Ruffatti A |title=Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases |journal=J. Rheumatol. |volume=36 |issue=8 |pages=1744–8 |date=August 2009 |pmid=19567621 |pmc=2798588 |doi=10.3899/jrheum.080737 |url=}}</ref>
*Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.<ref name="pmid19567621" /><ref name="pmid6982030">{{cite journal |vauthors=Bernstein RM, Bunn CC, Hughes GR |title=Identification of antibodies to acidic antigens by counterimmunoelectrophoresis |journal=Ann. Rheum. Dis. |volume=41 |issue=5 |pages=554–5 |date=October 1982 |pmid=6982030 |pmc=1001043 |doi=10.1136/ard.41.5.554 |url=}}</ref><ref name="pmid1985112">{{cite journal |vauthors=Chan EK, Hamel JC, Buyon JP, Tan EM |title=Molecular definition and sequence motifs of the 52-kD component of human SS-A/Ro autoantigen |journal=J. Clin. Invest. |volume=87 |issue=1 |pages=68–76 |date=January 1991 |pmid=1985112 |pmc=294993 |doi=10.1172/JCI115003 |url=}}</ref>
*Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.<ref name="pmid19567621" /><ref name="pmid6982030">{{cite journal |vauthors=Bernstein RM, Bunn CC, Hughes GR |title=Identification of antibodies to acidic antigens by counterimmunoelectrophoresis |journal=Ann. Rheum. Dis. |volume=41 |issue=5 |pages=554–5 |date=October 1982 |pmid=6982030 |pmc=1001043 |doi=10.1136/ard.41.5.554 |url=}}</ref><ref name="pmid1985112">{{cite journal |vauthors=Chan EK, Hamel JC, Buyon JP, Tan EM |title=Molecular definition and sequence motifs of the 52-kD component of human SS-A/Ro autoantigen |journal=J. Clin. Invest. |volume=87 |issue=1 |pages=68–76 |date=January 1991 |pmid=1985112 |pmc=294993 |doi=10.1172/JCI115003 |url=}}</ref>
===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].
*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].


== Treatment ==
== Treatment ==
Line 252: Line 239:
}}</ref>
}}</ref>
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for Congenital Heart Disease.
*There are no primary preventive measures available for Congenital Heart Disease.<ref name="JenkinsBotto2019">{{cite journal|last1=Jenkins|first1=Kathy J.|last2=Botto|first2=Lorenzo D.|last3=Correa|first3=Adolfo|last4=Foster|first4=Elyse|last5=Kupiec|first5=Jennifer K.|last6=Marino|first6=Bradley S.|last7=Oster|first7=Matthew E.|last8=Stout|first8=Karen K.|last9=Honein|first9=Margaret A.|title=Public Health Approach to Improve Outcomes for Congenital Heart Disease Across the Life Span|journal=Journal of the American Heart Association|volume=8|issue=8|year=2019|issn=2047-9980|doi=10.1161/JAHA.118.009450}}</ref>
*Fetus with second or third degree heart block are less in women who take HCQ.<ref name="TunksClowse2013">{{cite journal|last1=Tunks|first1=Robert D.|last2=Clowse|first2=Megan E.B.|last3=Miller|first3=Stephen G.|last4=Brancazio|first4=Leo R.|last5=Barker|first5=Piers C.A.|title=Maternal autoantibody levels in congenital heart block and potential prophylaxis with antiinflammatory agents|journal=American Journal of Obstetrics and Gynecology|volume=208|issue=1|year=2013|pages=64.e1–64.e7|issn=00029378|doi=10.1016/j.ajog.2012.09.020}}</ref><ref name="SaxenaIzmirly2015">{{cite journal|last1=Saxena|first1=Amit|last2=Izmirly|first2=Peter M.|last3=Han|first3=Sung Won|last4=Briassouli|first4=Paraskevi|last5=Rivera|first5=Tania L.|last6=Zhong|first6=Hua|last7=Friedman|first7=Deborah M.|last8=Clancy|first8=Robert M.|last9=Buyon|first9=Jill P.|title=Serum Biomarkers of Inflammation, Fibrosis, and Cardiac Function in Facilitating Diagnosis, Prognosis, and Treatment of Anti-SSA/Ro–Associated Cardiac Neonatal Lupus|journal=Journal of the American College of Cardiology|volume=66|issue=8|year=2015|pages=930–939|issn=07351097|doi=10.1016/j.jacc.2015.06.1088}}</ref>


*Women of childbearing age with systemic autoimmune disorders should be tested fo anti-Ro and anti-La antibodies.<ref>{{Cite journal
*Women of childbearing age with systemic autoimmune disorders should be tested fo anti-Ro and anti-La antibodies.<ref>{{Cite journal
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  | pmid = 25800217
  | pmid = 25800217
}}</ref>
}}</ref>
==References==
{{Reflist|2}}




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==References==
==References==
==References==
{{Reflist|2}}
{{refbegin|2}}
{{refbegin|2}}
*Figa FH, McCrindle BW, Bigras JL, et al. Risk factors for venous obstruction in children with transvenous pacing leads. Pacing Clin Electrophysiol. Aug 1997;20(8 Pt 1):1902-9.   
*Figa FH, McCrindle BW, Bigras JL, et al. Risk factors for venous obstruction in children with transvenous pacing leads. Pacing Clin Electrophysiol. Aug 1997;20(8 Pt 1):1902-9.   

Latest revision as of 01:49, 3 March 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2] Cafer Zorkun, M.D., Ph.D. [3]; Contributors: Adam C. Lake

Overview

Congenital heart block is a rare congenital heart disease caused by defects in the heart conduction system diagnosed on or before 28 days of life. It can lead to slowed heart rate.

Historical Perspective

  • Congenital heart block was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

  • Congenital Heart Block may be classified according to cause into 3 groups:[1]
  • First Degree Congenital Heart Block
  • Second Degree Congenital Heart Block
  • Third Degree Congenital (Complete) Heart Block

Pathophysiology

The normal physiology of congenital heart block can be understood as follows:[2][3][4][5]

  • Mother's antibodies cross from the fetus placenta and damage of cardiac conduction system. The majority of anti bodies are from mother to response an infectionin or in response to an autoimmune disorder (SLE).
  • Genetic disorde (autosomal recessive/dominant genetic trait).
  • In some patients, congenital heart block is a secondary characteristic disorders or heart muscle tumors (myocardium).
  • Fibrous tissue that either replaces the atrioventricular (AV) node and its surrounding tissue or by an interruption between the atrial myocardium and the AV nod.

Causes

The most common cause of congenital heart block is neonatal lupus.[6]

cardiac conduction system is damaged by antibodies that cross from the mother to the fetus via the placenta

It accounts for almost all cases presenting in the intrauterine and neonatal period. Other causes include:[7][8][9]

Differentiating [disease name] from other Diseases

Epidemiology and Demographics

  • The prevalence of congenital heart block is approximately 1 per 22,00 live births individuals worldwide. [12][13]
  • In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Congential heart block is diagnosed among patients aged from brith to 28 days old.
  • Congential heart block is observed among children.

Gender

  • Congenital heart block affects men and women equally.

Race

  • There is no racial predilection for congenital heart block.

Risk Factors

  • One form of congenital heart block occurs in babies whose mothers have autoimmune diseases, such as lupus.[14]
  • Patients with congenital heart block make proteins called antibodies that attack and damage the body's tissues or cells.[14]

Natural History, Complications, Prognosis

Prognosis

The damaged heart may beat extremely slowly. In some cases, the heart rate is so slow that it is fatal in nearly 20% of affected babies (with most deaths occurring as fetal demises). Patients presenting as fetuses or at birth have significantly higher morbidity and mortality rates than do patients presenting later in childhood.


Diagnosis


Symptoms

Physical Examination

  • Annular lesions (Face, particularly around eyes)
  • Photosensitive

Laboratory Findings

  • There are no specific laboratory findings associated with congenital heart block.[22]
  • May be autoantibodies to Ro/SSA and La/SSB ribonucleoproteins using ELISAW are helpful when when congenital heart block detected.[23]
  • Other laboratory findings consistent with the diagnosis of congenital heart block include Sera, ELISA, counter-immunoelectrophoresis (CIE) method.[23][24][25]

Treatment

Medical Therapy

Surgery

Prevention

  • There are no primary preventive measures available for Congenital Heart Disease.[35]
  • Fetus with second or third degree heart block are less in women who take HCQ.[36][37]


Electrocardiogram

Electrocardiographic findings in congenital heart block depend on the type of block.

Treatment

Surgery

Treatment depends on the type of heart block.

Nearly all surviving children with congenital heart block require permanent implantation of an pacemaker device.

Prevention

Because it is so difficult to treat or repair the damaged heart, a high-priority strategy is to try to prevent the inflammatory process before irreversible scarring can occur. The mother of the child should consult with a rheumatologist to begin monitoring for possible autoimmune disease. Consultation with a rheumatologist is also advised for the infant, particularly if other manifestations of neonatal lupus erythematosus are present. Genetic consultation is recommended for children with first-degree relatives with structural heart disease or those with storage disorder or cardiomyopathy.

Source

Related Chapters

References

References

  1. ↑ Baruteau, Alban-Elouen; Pass, Robert H.; Thambo, Jean-Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J. (2016). "Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management". European Journal of Pediatrics. 175 (9): 1235–1248. doi:10.1007/s00431-016-2748-0. ISSN 0340-6199.
  2. ↑ Zhou KY, Hua YM (December 2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chin. Med. J. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMC 5717867. PMID 29176145.
  3. ↑ 3.0 3.1 Fahed AC, Gelb BD, Seidman JG, Seidman CE (February 2013). "Genetics of congenital heart disease: the glass half empty". Circ. Res. 112 (4): 707–20. doi:10.1161/CIRCRESAHA.112.300853. PMC 3827691. PMID 23410880.
  4. ↑ Capone C, Buyon JP, Friedman DM, Frishman WH (2012). "Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population". Cardiol Rev. 20 (2): 72–6. doi:10.1097/CRD.0b013e31823c808b. PMC 3275696. PMID 22183063.
  5. ↑ Sharma, Gaurav; Linden, Michael D.; Schultz, Daniel S.; Inamdar, Kedar V. (2010). "Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart". Cardiovascular Pathology. 19 (3): e75–e78. doi:10.1016/j.carpath.2008.10.011. ISSN 1054-8807.
  6. ↑ Vinet, Évelyne; Pineau, Christian A.; Scott, Susan; Clarke, Ann E.; Platt, Robert W.; Bernatsky, Sasha (2015). "Increased Congenital Heart Defects in Children Born to Women With Systemic Lupus Erythematosus". Circulation. 131 (2): 149–156. doi:10.1161/CIRCULATIONAHA.114.010027. ISSN 0009-7322.
  7. ↑ Zhou KY, Hua YM (December 2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chin. Med. J. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMC 5717867. PMID 29176145.
  8. ↑ Lee LA (2004). "Neonatal lupus: clinical features and management". Paediatr Drugs. 6 (2): 71–8. doi:10.2165/00148581-200406020-00001. PMID 15035648.
  9. ↑ Yousuf T, Kramer J, Jones B, Keshmiri H, Dia M (2016). "Pulmonary Hypertension in a Patient With Congenital Heart Defects and Heterotaxy Syndrome". Ochsner J. 16 (3): 309–11. PMC 5024815. PMID 27660582.
  10. ↑ Capone C, Buyon JP, Friedman DM, Frishman WH (2012). "Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population". Cardiol Rev. 20 (2): 72–6. doi:10.1097/CRD.0b013e31823c808b. PMC 3275696. PMID 22183063.
  11. ↑ Lipshultz, Steven E.; Law, Yuk M.; Asante-Korang, Alfred; Austin, Eric D.; Dipchand, Anne I.; Everitt, Melanie D.; Hsu, Daphne T.; Lin, Kimberly Y.; Price, Jack F.; Wilkinson, James D.; Colan, Steven D. (2019). "Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association". Circulation. 140 (1). doi:10.1161/CIR.0000000000000682. ISSN 0009-7322.
  12. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  13. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  14. ↑ 14.0 14.1 Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  15. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  16. ↑ Hunter LE, Seale AN (September 2018). "EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Prenatal diagnosis of congenital heart disease". Echo Res Pract. 5 (3): R81–R100. doi:10.1530/ERP-18-0027. PMC 6107762. PMID 30012852.
  17. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  18. ↑ Donofrio, Mary T.; Moon-Grady, Anita J.; Hornberger, Lisa K.; Copel, Joshua A.; Sklansky, Mark S.; Abuhamad, Alfred; Cuneo, Bettina F.; Huhta, James C.; Jonas, Richard A.; Krishnan, Anita; Lacey, Stephanie; Lee, Wesley; Michelfelder, Erik C.; Rempel, Gwen R.; Silverman, Norman H.; Spray, Thomas L.; Strasburger, Janette F.; Tworetzky, Wayne; Rychik, Jack (2014). "Diagnosis and Treatment of Fetal Cardiac Disease". Circulation. 129 (21): 2183–2242. doi:10.1161/01.cir.0000437597.44550.5d. ISSN 0009-7322.
  19. ↑ 19.0 19.1 Kusumoto, Fred M.; Schoenfeld, Mark H.; Barrett, Coletta; Edgerton, James R.; Ellenbogen, Kenneth A.; Gold, Michael R.; Goldschlager, Nora F.; Hamilton, Robert M.; Joglar, José A.; Kim, Robert J.; Lee, Richard; Marine, Joseph E.; McLeod, Christopher J.; Oken, Keith R.; Patton, Kristen K.; Pellegrini, Cara N.; Selzman, Kimberly A.; Thompson, Annemarie; Varosy, Paul D. (2019). "2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay". Journal of the American College of Cardiology. 74 (7): e51–e156. doi:10.1016/j.jacc.2018.10.044. ISSN 0735-1097.
  20. ↑ Baruteau, Alban-Elouen; Pass, Robert H.; Thambo, Jean-Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J. (2016). "Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management". European Journal of Pediatrics. 175 (9): 1235–1248. doi:10.1007/s00431-016-2748-0. ISSN 0340-6199.
  21. ↑ Friedman D, Duncanson L, Glickstein J, Buyon J (July 2003). "A review of congenital heart block". Images Paediatr Cardiol. 5 (3): 36–48. PMC 3232542. PMID 22368629. Vancouver style error: initials (help)
  22. ↑ Brucato, Antonio; Grava, Chiara; Bortolati, Maria; Ikeda, Keigo; Milanesi, Ornella; Cimaz, Rolando; Ramoni, Veronique; Vignati, Gabriele; Martinelli, Stefano; Sadou, Youcef; Borghi, Adele; Tincani, Angela; Chan, Edward K.L.; Ruffatti, Amelia (2009). "Congenital Heart Block Not Associated with Anti-Ro/La Antibodies: Comparison with Anti-Ro/La-positive Cases". The Journal of Rheumatology. 36 (8): 1744–1748. doi:10.3899/jrheum.080737. ISSN 0315-162X.
  23. ↑ 23.0 23.1 Brucato A, Grava C, Bortolati M, Ikeda K, Milanesi O, Cimaz R, Ramoni V, Vignati G, Martinelli S, Sadou Y, Borghi A, Tincani A, Chan EK, Ruffatti A (August 2009). "Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases". J. Rheumatol. 36 (8): 1744–8. doi:10.3899/jrheum.080737. PMC 2798588. PMID 19567621.
  24. ↑ Bernstein RM, Bunn CC, Hughes GR (October 1982). "Identification of antibodies to acidic antigens by counterimmunoelectrophoresis". Ann. Rheum. Dis. 41 (5): 554–5. doi:10.1136/ard.41.5.554. PMC 1001043. PMID 6982030.
  25. ↑ Chan EK, Hamel JC, Buyon JP, Tan EM (January 1991). "Molecular definition and sequence motifs of the 52-kD component of human SS-A/Ro autoantigen". J. Clin. Invest. 87 (1): 68–76. doi:10.1172/JCI115003. PMC 294993. PMID 1985112.
  26. ↑ Clowse, Megan E B; Eudy, Amanda M; Kiernan, Elizabeth; Williams, Matthew R; Bermas, Bonnie; Chakravarty, Eliza; Sammaritano, Lisa R; Chambers, Christina D; Buyon, Jill (2018). "The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices". Rheumatology. 57 (suppl_5): v9–v17. doi:10.1093/rheumatology/key141. ISSN 1462-0324.
  27. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  28. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  29. ↑ Aurelie Ambrosi & Marie Wahren-Herlenius (2012). "Congenital heart block: evidence for a pathogenic role of maternal autoantibodies". Arthritis research & therapy. 14 (2): 208. doi:10.1186/ar3787. PMID 22546326. Unknown parameter |month= ignored (help)
  30. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  31. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  32. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  33. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  34. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  35. ↑ Jenkins, Kathy J.; Botto, Lorenzo D.; Correa, Adolfo; Foster, Elyse; Kupiec, Jennifer K.; Marino, Bradley S.; Oster, Matthew E.; Stout, Karen K.; Honein, Margaret A. (2019). "Public Health Approach to Improve Outcomes for Congenital Heart Disease Across the Life Span". Journal of the American Heart Association. 8 (8). doi:10.1161/JAHA.118.009450. ISSN 2047-9980.
  36. ↑ Tunks, Robert D.; Clowse, Megan E.B.; Miller, Stephen G.; Brancazio, Leo R.; Barker, Piers C.A. (2013). "Maternal autoantibody levels in congenital heart block and potential prophylaxis with antiinflammatory agents". American Journal of Obstetrics and Gynecology. 208 (1): 64.e1–64.e7. doi:10.1016/j.ajog.2012.09.020. ISSN 0002-9378.
  37. ↑ Saxena, Amit; Izmirly, Peter M.; Han, Sung Won; Briassouli, Paraskevi; Rivera, Tania L.; Zhong, Hua; Friedman, Deborah M.; Clancy, Robert M.; Buyon, Jill P. (2015). "Serum Biomarkers of Inflammation, Fibrosis, and Cardiac Function in Facilitating Diagnosis, Prognosis, and Treatment of Anti-SSA/Ro–Associated Cardiac Neonatal Lupus". Journal of the American College of Cardiology. 66 (8): 930–939. doi:10.1016/j.jacc.2015.06.1088. ISSN 0735-1097.
  38. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  39. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  40. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  41. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
  42. ↑ Kai-Yu Zhou & Yi-Min Hua (2017). "Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life". Chinese medical journal. 130 (23): 2863–2871. doi:10.4103/0366-6999.219160. PMID 29176145. Unknown parameter |month= ignored (help)
  43. ↑ Dm Friedman, Lj Duncanson, J. Glickstein & Jp Buyon (2003). "A review of congenital heart block". Images in paediatric cardiology. 5 (3): 36–48. PMID 22368629. Unknown parameter |month= ignored (help)
  44. ↑ Pilar Brito-Zeron, Peter M. Izmirly, Manuel Ramos-Casals, Jill P. Buyon & Munther A. Khamashta (2015). "The clinical spectrum of autoimmune congenital heart block". Nature reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. PMID 25800217. Unknown parameter |month= ignored (help)
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