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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]


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

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)
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:


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