Cyanosis surgery: Difference between revisions

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{{Cyanosis}}
{{Cyanosis}}
{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{Sara.Zand}}  


==Overview==
==Overview==
Cardiac defects causing [[central cyanosis]] include: [[Transposition of the great arteries]], [[Tetralogy of fallot]], [[Tricuspid atresia]], [[Truncus arteriosus]],[[Total anomalous pulmonary venous connection]], [[Ebstein anomaly]], critical [[Pulmonary stenosis]] or atresia, functional [[single ventricle]]. The palliative surgical shunt maybe done in such lesions to increase [[pulmonary blood flow]] even in the presence of [[cyanosis]]. Complete repair procedure leads to relief of cyanosis and shunt and also has long term complications.


== Cardiac catheterization ==
== Recommendation for surgery in [[cyanotic heart disease]] ==
* [[Cardiac catheter]] interventions can be palliative by improving cyanosis or be corrective by relieving obstruction to flow.
The table shows indications for surgery in [[cyanotic congenital heart disease]] according to 2018 [[AHA/ACC Guideline]]:<ref name="StoutDaniels2019">{{cite journal|last1=Stout|first1=Karen K.|last2=Daniels|first2=Curt J.|last3=Aboulhosn|first3=Jamil A.|last4=Bozkurt|first4=Biykem|last5=Broberg|first5=Craig S.|last6=Colman|first6=Jack M.|last7=Crumb|first7=Stephen R.|last8=Dearani|first8=Joseph A.|last9=Fuller|first9=Stephanie|last10=Gurvitz|first10=Michelle|last11=Khairy|first11=Paul|last12=Landzberg|first12=Michael J.|last13=Saidi|first13=Arwa|last14=Valente|first14=Anne Marie|last15=Van Hare|first15=George F.|title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines|journal=Circulation|volume=139|issue=14|year=2019|issn=0009-7322|doi=10.1161/CIR.0000000000000603}}</ref>
* [[Balloon valvuloplasty]] can be effective in patients with critical [[pulmonary stenosis]] or [[aortic stenosis]].
<ref name="ShiZhu2017">{{cite journal|last1=Shi|first1=Guocheng|last2=Zhu|first2=Zhongqun|last3=Chen|first3=Jimei|last4=Ou|first4=Yanqiu|last5=Hong|first5=Haifa|last6=Nie|first6=Zhiqiang|last7=Zhang|first7=Haibo|last8=Liu|first8=Xiaoqing|last9=Zheng|first9=Jinghao|last10=Sun|first10=Qi|last11=Liu|first11=Jinfen|last12=Chen|first12=Huiwen|last13=Zhuang|first13=Jian|title=Total Anomalous Pulmonary Venous Connection|journal=Circulation|volume=135|issue=1|year=2017|pages=48–58|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.116.023889}}</ref><ref>{{cite journal|doi=10.4103/2F0974-2069.157025}}</ref><ref name="Attenhofer JostConnolly2007">{{cite journal|last1=Attenhofer Jost|first1=Christine H.|last2=Connolly|first2=Heidi M.|last3=Dearani|first3=Joseph A.|last4=Edwards|first4=William D.|last5=Danielson|first5=Gordon K.|title=Ebstein’s Anomaly|journal=Circulation|volume=115|issue=2|year=2007|pages=277–285|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.106.619338}}</ref><ref name="pmid27275259">{{cite journal |vauthors=Idrizi S, Milev I, Zafirovska P, Tosheski G, Zimbakov Z, Ampova-Sokolov V, Angjuseva T, Mitrev Z |title=Interventional Treatment of Pulmonary Valve Stenosis: A Single Center Experience |journal=Open Access Maced J Med Sci |volume=3 |issue=3 |pages=408–12 |date=September 2015 |pmid=27275259 |pmc=4877828 |doi=10.3889/oamjms.2015.089 |url=}}</ref>
* Selected patients with [[pulmonary atresia]] are also candidates for [[balloon valvuloplasty]] if the obstruction is membranous.
== Pulmonary atresia ==
* [[Cardiac catheterization]] is done to evaluate the defect or defects of the heart; this procedure is much more invasive.
* The patient will need to have a series of surgeries to improve the blood flow permanently.
* The type of surgery recommended depends on the size of the [[right ventricle]] and the [[pulmonary artery]]. If they are normal in size and the [[right ventricle]] is able to pump blood, [[open heart surgery]] can be performed to make [[blood flow]] through the heart in a normal pattern.  
* If the [[right ventricle]] is small and unable to act as a pump, doctors may perform another type of operation called the [[Fontan procedure]]. In this two-stage procedure, the [[right atrium]] is disconnected from the [[pulmonary circulation]].  
* The systemic [[venous return]] goes directly to the lungs, by-passing the heart. The first surgery will likely be performed shortly after birth. A [[shunt]] can be created between the aorta and the [[pulmonary artery]] to help increase blood flow to the [[lungs]]. As the child grows, so does the heart and the [[shunt]] may need revised in order to meet the body's requirements.
* A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure as balloon atrial septostomy to improve mixing oxygenated blood and unoxygenated blood between the right and [[Left atrium|left atria]].
* '''Atrial Septostomy'''
* Aspecial catheter with a balloon in the tip is used to create an opening in the [[atrial septum]]. The catheter is guided through the [[foramen ovale]] to the [[left atrium]] (LA).  
* Once the ballon is in the LA is inflated and then pulled back opening a bigger hole between the right atrium and the LA to mix blood.
* If the hospital does not have a catheterization lab with skill physician to perform the ballon atrial septostomy, an intravenous medication called prostaglandin is administered to keep the [[Ductus arteriosus|ductus arteriousus]] from closing.


== Transposition of great arteries ==
'''Palliative interventions'''
*Cardiac catheterization
**Rashkind balloon atrial septostomy: A '''balloon atrial septostomy''' is performed with a balloon catheter, which is inserted into a foramen ovale, PFO, or ASD and inflated to enlarge the opening in the [[Interatrial septum|atrial septum;]] this creates a shunt which allows a larger amount of red blood to enter the systemic circulation.
**[[Balloon angioplasty]]: [[Angioplasty]] also requires a balloon catheter, which is used to stretch open a stenotic vessel; this relieves restricted blood flow, which could otherwise lead to [[Congestive heart failure|CHF]].
**Endovascular stenting: An '''endovascular stent''' is sometimes placed in a stenotic vessel immediately following a balloon angioplasty to maintain the widened passage.
**[[Angiography]]: Angiography involves using the catheter to release a contrast medium into the chambers and vessels of the heart; this process facilitates examining the flow of blood through the chambers during an [[Echocardiography|echocardiogram]], or shows the vessels clearly on a [[Chest X-ray|chest x-ray]], [[MRI]], or [[CT scan]] - this is of particular importance, as the coronary arteries must be carefully examined and "mapped out" prior to the corrective surgery. It is commonplace for any of these [[Palliation|palliations]] to be performed on a [[TGA]] patient.
'''Moderate'''
*Left [[Thoracotomy|anterior thoracotomy]]
*Left lateral thoracotomy
*Right lateral thoracotomy
*Each of these procedures are performed through an incision between the ribs and visualized by echocardiogram; these are far less common than heart cath procedures.
*Pulmonary artery banding is used in a small number of cases of d-TGA, usually when the corrective surgery needs to be delayed, to create an artificial stenosis in order to control pulmonary blood pressure; PAB involves placing a band around the pulmonary trunk, this band can then be quickly and easily adjusted when necessary.
*An atrial septectomy is the surgical removal of the atrial septum; this is performed when a foramen ovale, PFO, or ASD are not present and additional shunting is required to raise the oxygen saturation of the blood.
'''Major'''
*Median sternotomy
*PAB (when intracardiac procedures also required)
*Concomitant atrial septectomy
== Total anomalus pulmonary venous return ==
Surgery should be performed as soon as possible in the patients of total anomalous pulmonary venous connection. The surgical procedure varies depending upon the anatomy of the TAPVC lesion.
*Supracardiac and infracardiac TAPVC: Connection is created between the [[Pulmonary vein|pulmonary veins]] and the [[left atrium]]. The vertical vein is tied up so that the abnormal blood flow could be prevented.
*Pulmonary veins directly connected to the superiorvenacava: An intracardiac baffle is created that helps in transfer of blood from the [[right atrium]], through atrial septum into the left atrium.
*Intracardiac (pulmonary vein connected to the coronary sinus): Coronary sinus is incised and connected to left atrium.
*Intracardiac (pulmonary vein opening directly into the right atrium): A interatrial connection is made and the blood is redirected from right atrium to left atrium.


== Tricuspid atresia ==


== Tetralogy of fallot ==
'''<span style="font-size:85%">'''Abbreviations:'''
'''d-TGA:''' [[dextro-Transposition of great arteries]];
'''PDA:''' [[Patent ductus arteriosus]]  ;
'''ASD:''' [[Atrial septal defect]];
'''VSD:''' [[Ventricular septal defect]];
'''TAPVC:''' [[Total anomalous pulmonary venous connection]];
'''TOF:''' [[Tetralogy of fallot]];
'''CCTGA:''' [[Congenitally corrected transposition of the great arteries]];
'''PS:''' [[Pulmonary stenosis]];
'''AF:''' [[Atrial fibrillation]];
'''VF:''' [[Ventricular fibrillation]];
'''PR:''' [[Pulmonary regurgitation]];
'''RVOT:''' [[Right ventricular outflow tract]];
'''CMR:''' [[Cardiovascular magnetic resonance]];
'''SVC:''' [[Superior vena cava]];
'''IVC:''' [[Inferior vena cava]];
</span>
<br>


=== Palliative surgery ===
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
*The surgery involved forming an [[anastomosis]] between the [[subclavian artery]] and the [[pulmonary artery]].
|-
*The Pott shunt and the Waterson procedure are other shunt procedures which were developed for the same purpose.
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF;" align=center |''' Recommendation for surgery in [[cyanotic congenital heart disease]]'''
*Potts shunt: Shunt between [[left pulmonary artery]] and [[descending aorta]].
|-
*The technique has been modified and is usually performed using a Gortex tube to create the connection.
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left | ''' Indications for repair of a scimitar vein in [[Anomalous pulmonary venous connection]] ([[TAPVC]])  ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]] ):'''
*Waterson shunt: Shunt between [[right pulmonary artery]] and [[ascending aorta]].
|-
===Total Surgical Repair===
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
*The surgery generally involves:
❑ Decreased functional capacity <br> 
**Making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle
❑ [[Right ventricle]] enlagment <br>
**Repairing the VSD using a Gore-Tex or Dacron patch or a homograft.
❑ Net left to right shunt or QP/QS > 1.5/1<br>
**Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.
❑ [[Pulmonary artery ]]systolic pressure less than 50% systemic pressure<br>
**The repair could be done by either of the approaches i.e.transatrial or transpulmonary
❑ [[Pulmonary vascular resistance]] less than 1/3 of systemic resistance<br>
❑ Repair at the time of closure of a sinus venous defect or [[ASD]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] Abnormal connection between pulmonary veins and systemic veins leading to right heart volume overload such as ASD</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |''' Indications for [[surgery]] in  [[Anomalous Pulmonary Venous Connections]] ([[TAPVC]])'''([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]]) :'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic adults with [[right ventricle]] volume overload<br>
❑ Large left to right shunt( QP/QS > 1.5/1 <br>
❑ [[Pulmonary artery pressure]] <50% systemic pressure and [[pulmonary artery resistance]] <1/3 systemic resistance<br>
❑ Evidence of [[Right ventricle]] volume overload and QP/QS>1.5/1<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |''' Indications for [[surgical]] repair or reoperation  in [[Ebstein anomaly]] : ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Significant [[tricuspid regurgitation]] in the presence of the following:<br>
❑ [[Heart failure ]] symptoms<br>
❑ Decreased [[functional capacity]]<br>
❑ Progressive [[right ventricular]] dysfunction by [[echocardiography]] or [[cardiac MRI]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] Malformation of tricuspid valve and right ventricle , atrialization of right ventricle, huge right atrium, accompanied by ASD, VSD, PS</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indications for [[surgical]] repair or reoperation in [[Ebstein anomaly]]:([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Significant [[tricuspid regurgitation ]] in the presence of the following:<br>
❑ Progressive [[right ventricle]] enlargement<br>
❑ Systemic desaturation due to [[right to left shunt]] via [[ASD]], [[VSD]]<br>
❑ [[Paradoxical emboli]] through [[ASD]], [[VSD]]<br>
❑ [[Atrial tachycardia]]<br>
<span style="font-size:85%;color:red"> [[Other surgery procedures|<span style="color:red"> Other surgery procedures:</span>]] Closure ASD, ablation of multiple accessory pathway for prevention of VF, left atrium COX-MAZ 3  in the presence of AF</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for Glenn anastomousis at the time of repair in [[Ebstein anomaly]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Severe [[right ventricle]] dilation <br>
❑ Severe [[right ventricular systolic dysfunction]]<br>
❑ Normal [[left ventricle]] function<br>
❑ Normal [[left atrium]] or [[left ventricle end diastolic pressure]]<br>
<span style="font-size:85%;color:red"> [[Glenn shunt|<span style="color:red"> Glenn anastomosis:</span>]] Bidirectional superior cavopulmonary anastomosis which is the connection between superior vena cava and pulmonary artery with bypassing right artium and right ventricle, cardiac catheterization should be done before glenn anastomosis especially in adult with hypertension  for evaluation of left ventricle diastolic pressure. </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left | ''' Indication for [[pulmonary valve replacement ]] ( surgical or percutaneous in [[Tetralogy of fallot]] ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]]):'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑  Symptomatic Moderate to severe [[pulmonary regurgitation]] after repaired [[TOF]] in which symptoms can not be explained otherwise.<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] The combination of Right ventricle hypertrophy, VSD, PS, Overridding aorta</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Pulmonary valve replacement ]] ( surgical or percutaneous in [[Tetralogy of fallot]] : ([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic moderate to severe [[ pulmonary regurgitation]] after repaired [[TOF]]<br>
<span style="font-size:85%;color:red"> Pulmonary stenosis valvotomy:<span style="color:red"> Surgical or balloon valvotomy in case of severe PS during infancy or childhood is recommended. Both of interventions can result pulmonary regurgitation and right ventricle dilation  in the future and the need for pulmonary valve replacement.</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Pulmonary valve replacement ]] (surgical) in [[Tetralogy of fallot]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Moderate to severe [[PR]] with other lesions requiring surgery in repaired [[TOF]]<br>
❑ Moderate to severe [[PR]] with ventricular tachycardia requiring arrhythmia management in repaired [[TOF]]<br>
<span style="font-size:85%;color:red"> [[Other lesions requiring surgery|<span style="color:red"> Other lesions requiring surgery:</span>]] RVOT aneurysm, TR, branch PA stenosis, residual VSD, arrhythmia ablation, coronary artery revascularization, aortic root replacement </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Tricuspid valve replacement ]] in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Symptomatic severe [[tricuspid regurgitaion]] accompanied by preserved or mildly systolic dysfunction of systemic ventricle<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Tricuspid valve replacement ]] in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic  severe [[tricuspid regurgitation]] accompanied by mildly dilated systemic [[ventricle]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red"> Definition:</span>]] Atrioventricular discordance and ventriculoarterial discordance leading to physiologic corrected circulation accompanied by VSD(75%), pulmonary or subpulmonary stenosis(75%),left sided tricuspid and Ebstein like valve anomalies(75%) </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for conduit intervention/replacement in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Symptomatic subpulmonary [[left ventricle]] to [[pulmonary artery]] conduit dysfunction <br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Balloon valvoplasty ]] in  [[Pulmonary stenosis]] : ([[ACC AHA guidelines classification scheme|Class I , Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ In adult with moderate to severe  [[valvular PS]] with symptoms of [[heart failure]], [[cyanosis]] from intracardiac  right to left [[shunt]], [[exercise intolerance]]<br>
❑ Surgical repair is recommended if balloon valvoplasty  in indicated patients failed <br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Balloon valvoplasty]] in  [[Pulmonary Stenosis]] : ([[ACC AHA guidelines classification scheme|Class 2a , Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic severe [[valvular PS]]<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Dilation and Stenting]] in [[Peripheral Pulmonary Stenosis]] : ([[ACC AHA guidelines classification scheme|Class  2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ In an adult with branch and peripheral [[PS]], [[pulmonary artery]] dilation  and stenting is recommended
|}


== Coarcitation of aorta ==
== Surgical procedures in [[d-TGA]] and [[Tricuspid Atresia]] ==


===Indications for Surgery===
*There is a lack of consensus for the indications and the time for surgery. Some groups of surgeon consider [[balloon angioplasty]] and stenting as the initial approach and reserving surgery for more complicated conditions or treatment failures. Whereas, others consider surgical repair as the first line of therapy.
*Surgery is the first choice in aortic coarctations involving:
**Long length of aorta
**Severe coarctations causing almost complete or complete obliteration of the aortic lumen.
**Associated with other cardiac defects for e.g. large [[patent ductus arteriosus]], [[ventricular septal defect]].
=== Surgical Techniques===
*The choice of technique depends on the patient's age at presentation, size, associated abnormalities, and anatomy of the coarctation.
*Surgical approach - [[median sternotomy]] is preferred over left lateral thoracotomy, in complex arch repairs.
*Subclavian flap aortoplasty is found to be the most commonly performed followed by resection in end-to-end anastomosis, patch aortoplasty, and bypass graft when the surgery is done during infancy. Whereas, in children and adolescent undergoing coarctation repair end-to-end anastomosis is commonest followed patch aortoplasty and subclavian flap aortoplasty.
*Patch aortoplasty are less frequently used these days because of concerns regarding the development of aortic aneurysm at the site of surgery. 
*Available Techniques:
**Resection and end-to-end anastomosis
**Patch aortoplasty
**Left subclavian flap aortoplasty
**Tubular bypass grafts
**Combination techniques 


== Atrial septal defect ==
{| class="wikitable"
*Surgical closure is the most common method of treatment method for [[atrial septal defect]] and has been the gold standard for many years. Many surgeons prefer more [[Minimally invasive surgery|minimally invasive techniques]] over the conventional [[sternotomy]] to avoid potentials for additional complications. Special consideration must be taken into account for the age of the patient and the size of the defect involved. Surgical closure is indicated for patients with [[Atrial septal defect ostium primum|primum]], [[Atrial septal defect sinus venosus|sinus venosus]] and [[Atrial septal defect coronary sinus|coronary sinus]] type of [[Atrial septal defect|atrial septal defects]]. However, ostium secundum atrial septal defects are commonly treated by percutaneous closure. With uncomplicated [[atrial septal defect]], (without [[pulmonary hypertension]] and other comorbidities) the post-surgical mortality is as low as 1%.  Minimally invasive repair of [[atrial septal defect]] has been shown to be as successful as the conventional [[sternotomy]]. Although they have not been associated with reduced morbidity and mortality rates, they have been proven to have the advantage of being less invasive, less post-surgical complications, decreased hospital stay, and more cosmetic benefits.
|-
! Surgical management in [[d-TGA]] || Surgical management in [[Tricuspid Atresia]]
|-
! Life-saving balloon atrial septostomy in neonatal period||Blalock Taussig shunt
|-
|Connection  between right and the left system is necessary for the life
||                                                   
*In the first 8 weeks of birth  for transferring the systemic  blood to the pulmonary circulation in the neonate with cyanosia and pulmonary obstruction and normal positioning [[aorta]] and [[pulmonary artery]]<ref name="pmid26260095">{{cite journal |vauthors=Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G |title=Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease |journal=Pediatr Cardiol |volume=37 |issue=1 |pages=131–4 |date=January 2016 |pmid=26260095 |doi=10.1007/s00246-015-1251-0 |url=}}</ref><span style="font-size:85%;color:red"> [[Definition|<span style="color:red"> Definition:</span>]] Absence of trisuspid valve and right ventricle hypoplasia,connection via ASD is necessary </span><br>
|-
! Atrial switch procedure|| [[Pulmonary artery]] banding.<ref name="pmid30811802">{{cite journal |vauthors=Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC |title=Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature |journal=Congenit Heart Dis |volume=14 |issue=1 |pages=105–109 |date=January 2019 |pmid=30811802 |doi=10.1111/chd.12707 |url=}}</ref>
|-
| 
* Mustard  procedure (baffle made of Dacron or [[pericardium]]) or [[Senning]] procedure(atrial flap)<ref name="VejlstrupSørensen2015">{{cite journal|last1=Vejlstrup|first1=Niels|last2=Sørensen|first2=Keld|last3=Mattsson|first3=Eva|last4=Thilén|first4=Ulf|last5=Kvidal|first5=Per|last6=Johansson|first6=Bengt|last7=Iversen|first7=Kasper|last8=Søndergaard|first8=Lars|last9=Dellborg|first9=Mikael|last10=Eriksson|first10=Peter|title=Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark|journal=Circulation|volume=132|issue=8|year=2015|pages=633–638|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.114.010770}}</ref>
 
* The most common procedure in older patients
* Blood is diverted at the atrial level into the physiologic ventricle
* Systemic venous return is diverted into [[left ventricle]] through [[mitral valve]]
* Pulmonary venous return is directed into [[right ventricle]] through [[tricuspid valve]]
<span style="font-size:85%;color:red"> [[d-TGA definition|<span style="color:red"> d-TGA definition:</span>]] Aorta arises  from right ventricle and pulmonary artery arises from left ventricle </span><br>
||
* Useful in  overflow [[pulmonary artery]] coming from [[left ventricle]] for lowering the [[pulmonary blood flow]]
|-
! Arterial switch procedure || Bidirectional Glenn shunt
|-
|                           
*Transection and anastomoses of [[aorta]] and [[pulmonary artery ]] to the contralateral ventricle
* Closure of the [[VSD]]
* Transposioning of the [[coronary artrie]]
* Benefit is the restoration of [[left ventricular]] systolic function
||
*Useful in older children for blood transferring from [[SVC]] to [[pulmonary artery]]
|-
! Rastelli procedure|| Fontan procedure<ref name="pmid8238751">{{cite journal |vauthors=Norwood WI, Jacobs ML |title=Fontan's procedure in two stages |journal=Am. J. Surg. |volume=166 |issue=5 |pages=548–51 |date=November 1993 |pmid=8238751 |doi=10.1016/s0002-9610(05)81151-1 |url=}}</ref><ref name="d'UdekemIyengar2007">{{cite journal|last1=d'Udekem|first1=Y.|last2=Iyengar|first2=A. J.|last3=Cochrane|first3=A. D.|last4=Grigg|first4=L. E.|last5=Ramsay|first5=J. M.|last6=Wheaton|first6=G. R.|last7=Penny|first7=D. J.|last8=Brizard|first8=C. P.|title=The Fontan Procedure: Contemporary Techniques Have Improved Long-Term Outcomes|journal=Circulation|volume=116|issue=11_suppl|year=2007|pages=I-157–I-164|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.106.676445}}</ref>
 
 
|-
|
* Systemic to pulmonary artery shunt when [[pulmonary blood flow]] decreases<ref>{{cite journal|doi=10.1177/2F2150135118817765}}</ref>
 
* Useful in infants with [[TGA]] and [[VSD]] and [[PS]]
*Function: Re-direction of ventricular outflow
*Placement a conduit between [[right ventricle]] and [[pulmonary artery]] and also a baffle between [[left ventricle]] and [[aorta]]
* Survival rate is poor compared with mustard and arterial switch
||
*Useful in older children aged 2-3
* A conduit for transferring  blood from [[IVC]] to [[pulmonary artery]]
|-
|}
 
== Palliative Systemic-to-Pulmonary shunts ==
{| class="wikitable"
|-
! Arterial
|-
| [[Blalock-taussing-Thomas shunt]] ([[subclavian artery]] to [[pulmonary artery]])
|-
| [[Central shunt]] ([[aorta]] to [[pulmonary artery]])
|-
| [[Potts shunt]] (descending aorta to [[left pulmonary artery]]
|-
| [[Waterston shunt]] ([[Ascending aorta]] to [[right pulmonary artery]])
|-
! Venous
|-
| Glenn shunt ([[SVC]] to the ipsilateral [[pulmonary artery]])
|-
| Bidirectional cavopulmonary (Glenn) shunt( end to side [[SVC]]  to [[left pulmonary artery]]  and [[right pulmonary artery]] shunt)
|}
 
* [[Right ventricle]]–to-[[pulmonary artery]] conduits is recommended in severe [[RVOT]] obstruction such as [[pulmonary atresia]].  
* theses conduits may be homografts or prosthetic conduits with bioprosthetic valves using within the conduit.
*Common complications of the conduits may include the following:
:*Kinking
:*[[Aneurysmal dilation]]
:*Conduit dysfunction over time
:*Progressive stenosis within the [[conduit]] or at the [[valve]]
:*Valvular [[regurgitation]]


== Pulmonary hypertension ==
*The choice of treatment for pulmonary hypertension requires the assessment of the clinical severity of the disease and the identification of any underlying cause.
*Patients who have PH secondary to a medical condition such as [[left heart failure]], [[lung]] diseases, or [[Thromboembolism|thromboembolic disease]] should receive treatment for the underlying cause.
*Patients who have pulmonary arterial hypertension (PAH) must undergo vasoreactivity testing in order to assist in the selection of the optimal therapy which includes [[Calcium channel blocker|calcium channel blockers]], [[endothelin receptor antagonist]], [[Phosphodiesterase inhibitor|phosphodiesterase inhibitors]], or [[Prostanoid|prostanoids]].
*Surgical intervention such as [[atrial septostomy]] or [[lung transplantation]] should be considered among patients with pulmonary arterial hypertension who fail to improve on optimal therapy or when medical therapy is unavailable.
*Failure of clinical improvement among PAH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class II or III]] is defined as either:
**A stable and unsatisfactory clinical status, or
**An unstable and deteriorating Failure of clinical improvement among PAH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] is defined as either:
**Absence of quick improvement to a WHO functional class III or less, or
**A stable and unsatisfactory clinical status
===Atrial Septostomy===
*[[Atrial septostomy]] is a surgical procedure that creates a shunt between the right and left [[atria]].
*It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood ([[hypoxia]]). It is best performed in experienced centers.
*Graded balloon dilatation of atrial septostomy is the recommended type of [[atrial septostomy]].
*Atrial septostomy might be considered among PH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] and [[right heart failure]] refractory to therapy.
*[[Atrial septostomy]] should also be considered among PH patients who have [[Eisenmenger's syndrome]], idiopathic PAH, and those awaiting [[lung transplantation]].
===Lung Transplantation===
*[[Lung transplantation]] is considered in the treatment of patients with idiopathic PH, PH associated with [[congenital heart disease]], or pulmonary veno-occlusive disease (PVOD) who fail to improve on optimal medical therapy.
*Combined lung and heart transplantation might be considered in selected patients.
*According to the Registry of the International Society for Heart and Lung Transplantation, the survival rates following [[lung transplantation]] are 61%, 49%, and 25 % at 3, 5, and 10 years respectively.
===Pulmonary Thromboendarterectomy===
*[[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for the treatment of chronic thromboembolic pulmonary hypertension.
*It is the surgical removal of an organized [[thrombus]] along with the lining of the [[pulmonary artery]].
*PTE is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
*Treatment for hypoxic and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
 
[[Category:Up To Date]]
{{WH}}
{{WS}}
[[Category: (name of the system)]]

Latest revision as of 14:24, 4 March 2021

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Zand, M.D.[2]

Overview

Cardiac defects causing central cyanosis include: Transposition of the great arteries, Tetralogy of fallot, Tricuspid atresia, Truncus arteriosus,Total anomalous pulmonary venous connection, Ebstein anomaly, critical Pulmonary stenosis or atresia, functional single ventricle. The palliative surgical shunt maybe done in such lesions to increase pulmonary blood flow even in the presence of cyanosis. Complete repair procedure leads to relief of cyanosis and shunt and also has long term complications.

Recommendation for surgery in cyanotic heart disease

The table shows indications for surgery in cyanotic congenital heart disease according to 2018 AHA/ACC Guideline:[1] [2][3][4][5]


Abbreviations: d-TGA: dextro-Transposition of great arteries; PDA: Patent ductus arteriosus  ; ASD: Atrial septal defect; VSD: Ventricular septal defect; TAPVC: Total anomalous pulmonary venous connection; TOF: Tetralogy of fallot; CCTGA: Congenitally corrected transposition of the great arteries; PS: Pulmonary stenosis; AF: Atrial fibrillation; VF: Ventricular fibrillation; PR: Pulmonary regurgitation; RVOT: Right ventricular outflow tract; CMR: Cardiovascular magnetic resonance; SVC: Superior vena cava; IVC: Inferior vena cava;

Recommendation for surgery in cyanotic congenital heart disease
Indications for repair of a scimitar vein in Anomalous pulmonary venous connection (TAPVC) (Class I, Level of Evidence B ):

❑ Decreased functional capacity
❑ Right ventricle enlagment
❑ Net left to right shunt or QP/QS > 1.5/1
❑ Pulmonary artery systolic pressure less than 50% systemic pressure
❑ Pulmonary vascular resistance less than 1/3 of systemic resistance
❑ Repair at the time of closure of a sinus venous defect or ASD
Definition: Abnormal connection between pulmonary veins and systemic veins leading to right heart volume overload such as ASD

Indications for surgery in Anomalous Pulmonary Venous Connections (TAPVC)(Class 2a, Level of Evidence B) :

❑ Asymptomatic adults with right ventricle volume overload
❑ Large left to right shunt( QP/QS > 1.5/1
❑ Pulmonary artery pressure <50% systemic pressure and pulmonary artery resistance <1/3 systemic resistance
❑ Evidence of Right ventricle volume overload and QP/QS>1.5/1

Indications for surgical repair or reoperation in Ebstein anomaly : (Class I, Level of Evidence B)

❑ Significant tricuspid regurgitation in the presence of the following:
❑ Heart failure symptoms
❑ Decreased functional capacity
❑ Progressive right ventricular dysfunction by echocardiography or cardiac MRI
Definition: Malformation of tricuspid valve and right ventricle , atrialization of right ventricle, huge right atrium, accompanied by ASD, VSD, PS

Indications for surgical repair or reoperation in Ebstein anomaly:(Class 2a, Level of Evidence B)

❑ Significant tricuspid regurgitation in the presence of the following:
❑ Progressive right ventricle enlargement
❑ Systemic desaturation due to right to left shunt via ASD, VSD
❑ Paradoxical emboli through ASD, VSD
❑ Atrial tachycardia
Other surgery procedures: Closure ASD, ablation of multiple accessory pathway for prevention of VF, left atrium COX-MAZ 3 in the presence of AF

Indication for Glenn anastomousis at the time of repair in Ebstein anomaly : (Class 2b, Level of Evidence B)

❑ Severe right ventricle dilation
❑ Severe right ventricular systolic dysfunction
❑ Normal left ventricle function
❑ Normal left atrium or left ventricle end diastolic pressure
Glenn anastomosis: Bidirectional superior cavopulmonary anastomosis which is the connection between superior vena cava and pulmonary artery with bypassing right artium and right ventricle, cardiac catheterization should be done before glenn anastomosis especially in adult with hypertension for evaluation of left ventricle diastolic pressure.

Indication for pulmonary valve replacement ( surgical or percutaneous in Tetralogy of fallot (Class I, Level of Evidence B):

❑ Symptomatic Moderate to severe pulmonary regurgitation after repaired TOF in which symptoms can not be explained otherwise.
Definition: The combination of Right ventricle hypertrophy, VSD, PS, Overridding aorta

Indication for Pulmonary valve replacement ( surgical or percutaneous in Tetralogy of fallot : (Class 2a, Level of Evidence B)

❑ Asymptomatic moderate to severe pulmonary regurgitation after repaired TOF
Pulmonary stenosis valvotomy: Surgical or balloon valvotomy in case of severe PS during infancy or childhood is recommended. Both of interventions can result pulmonary regurgitation and right ventricle dilation in the future and the need for pulmonary valve replacement.

Indication for Pulmonary valve replacement (surgical) in Tetralogy of fallot : (Class 2b, Level of Evidence C)

❑ Moderate to severe PR with other lesions requiring surgery in repaired TOF
❑ Moderate to severe PR with ventricular tachycardia requiring arrhythmia management in repaired TOF
Other lesions requiring surgery: RVOT aneurysm, TR, branch PA stenosis, residual VSD, arrhythmia ablation, coronary artery revascularization, aortic root replacement

Indication for Tricuspid valve replacement in CCTGA : (Class I, Level of Evidence B)

❑ Symptomatic severe tricuspid regurgitaion accompanied by preserved or mildly systolic dysfunction of systemic ventricle

Indication for Tricuspid valve replacement in CCTGA : (Class 2a, Level of Evidence C)

❑ Asymptomatic severe tricuspid regurgitation accompanied by mildly dilated systemic ventricle
Definition: Atrioventricular discordance and ventriculoarterial discordance leading to physiologic corrected circulation accompanied by VSD(75%), pulmonary or subpulmonary stenosis(75%),left sided tricuspid and Ebstein like valve anomalies(75%)

Indication for conduit intervention/replacement in CCTGA : (Class 2b, Level of Evidence B)

❑ Symptomatic subpulmonary left ventricle to pulmonary artery conduit dysfunction

Indication for Balloon valvoplasty in Pulmonary stenosis : (Class I , Level of Evidence B)

❑ In adult with moderate to severe valvular PS with symptoms of heart failure, cyanosis from intracardiac right to left shunt, exercise intolerance
❑ Surgical repair is recommended if balloon valvoplasty in indicated patients failed

Indication for Balloon valvoplasty in Pulmonary Stenosis : (Class 2a , Level of Evidence C)

❑ Asymptomatic severe valvular PS

Indication for Dilation and Stenting in Peripheral Pulmonary Stenosis : (Class 2a, Level of Evidence B)

❑ In an adult with branch and peripheral PS, pulmonary artery dilation and stenting is recommended

Surgical procedures in d-TGA and Tricuspid Atresia

Surgical management in d-TGA Surgical management in Tricuspid Atresia
Life-saving balloon atrial septostomy in neonatal period Blalock Taussig shunt
Connection between right and the left system is necessary for the life
  • In the first 8 weeks of birth for transferring the systemic blood to the pulmonary circulation in the neonate with cyanosia and pulmonary obstruction and normal positioning aorta and pulmonary artery[6] Definition: Absence of trisuspid valve and right ventricle hypoplasia,connection via ASD is necessary
Atrial switch procedure Pulmonary artery banding.[7]

d-TGA definition: Aorta arises from right ventricle and pulmonary artery arises from left ventricle

Arterial switch procedure Bidirectional Glenn shunt
Rastelli procedure Fontan procedure[9][10]


  • Useful in older children aged 2-3
  • A conduit for transferring blood from IVC to pulmonary artery

Palliative Systemic-to-Pulmonary shunts

Arterial
Blalock-taussing-Thomas shunt (subclavian artery to pulmonary artery)
Central shunt (aorta to pulmonary artery)
Potts shunt (descending aorta to left pulmonary artery
Waterston shunt (Ascending aorta to right pulmonary artery)
Venous
Glenn shunt (SVC to the ipsilateral pulmonary artery)
Bidirectional cavopulmonary (Glenn) shunt( end to side SVC to left pulmonary artery and right pulmonary artery shunt)
  • Right ventricle–to-pulmonary artery conduits is recommended in severe RVOT obstruction such as pulmonary atresia.
  • theses conduits may be homografts or prosthetic conduits with bioprosthetic valves using within the conduit.
  • Common complications of the conduits may include the following:

References

  1. ↑ Stout, Karen K.; Daniels, Curt J.; Aboulhosn, Jamil A.; Bozkurt, Biykem; Broberg, Craig S.; Colman, Jack M.; Crumb, Stephen R.; Dearani, Joseph A.; Fuller, Stephanie; Gurvitz, Michelle; Khairy, Paul; Landzberg, Michael J.; Saidi, Arwa; Valente, Anne Marie; Van Hare, George F. (2019). "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines". Circulation. 139 (14). doi:10.1161/CIR.0000000000000603. ISSN 0009-7322.
  2. ↑ Shi, Guocheng; Zhu, Zhongqun; Chen, Jimei; Ou, Yanqiu; Hong, Haifa; Nie, Zhiqiang; Zhang, Haibo; Liu, Xiaoqing; Zheng, Jinghao; Sun, Qi; Liu, Jinfen; Chen, Huiwen; Zhuang, Jian (2017). "Total Anomalous Pulmonary Venous Connection". Circulation. 135 (1): 48–58. doi:10.1161/CIRCULATIONAHA.116.023889. ISSN 0009-7322.
  3. ↑ . doi:10.4103/2F0974-2069.157025. Missing or empty |title= (help)
  4. ↑ Attenhofer Jost, Christine H.; Connolly, Heidi M.; Dearani, Joseph A.; Edwards, William D.; Danielson, Gordon K. (2007). "Ebstein's Anomaly". Circulation. 115 (2): 277–285. doi:10.1161/CIRCULATIONAHA.106.619338. ISSN 0009-7322.
  5. ↑ Idrizi S, Milev I, Zafirovska P, Tosheski G, Zimbakov Z, Ampova-Sokolov V, Angjuseva T, Mitrev Z (September 2015). "Interventional Treatment of Pulmonary Valve Stenosis: A Single Center Experience". Open Access Maced J Med Sci. 3 (3): 408–12. doi:10.3889/oamjms.2015.089. PMC 4877828. PMID 27275259.
  6. ↑ Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G (January 2016). "Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease". Pediatr Cardiol. 37 (1): 131–4. doi:10.1007/s00246-015-1251-0. PMID 26260095.
  7. ↑ Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC (January 2019). "Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature". Congenit Heart Dis. 14 (1): 105–109. doi:10.1111/chd.12707. PMID 30811802.
  8. ↑ Vejlstrup, Niels; Sørensen, Keld; Mattsson, Eva; Thilén, Ulf; Kvidal, Per; Johansson, Bengt; Iversen, Kasper; Søndergaard, Lars; Dellborg, Mikael; Eriksson, Peter (2015). "Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark". Circulation. 132 (8): 633–638. doi:10.1161/CIRCULATIONAHA.114.010770. ISSN 0009-7322.
  9. ↑ Norwood WI, Jacobs ML (November 1993). "Fontan's procedure in two stages". Am. J. Surg. 166 (5): 548–51. doi:10.1016/s0002-9610(05)81151-1. PMID 8238751.
  10. ↑ d'Udekem, Y.; Iyengar, A. J.; Cochrane, A. D.; Grigg, L. E.; Ramsay, J. M.; Wheaton, G. R.; Penny, D. J.; Brizard, C. P. (2007). "The Fontan Procedure: Contemporary Techniques Have Improved Long-Term Outcomes". Circulation. 116 (11_suppl): I-157–I-164. doi:10.1161/CIRCULATIONAHA.106.676445. ISSN 0009-7322.
  11. ↑ . doi:10.1177/2F2150135118817765. Missing or empty |title= (help)
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