Cyanosis surgery

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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 defect 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:

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;

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

• • In the first 8 weeks of life if there are severe Cyanosis and pulmonary obstruction and normal positioning aorta and pulmonary artery, making a shunt between systemic subclavian artery to the pulmonary artery is necessary which is called Blalock -Taussig (BT shunt).
  • If the pulmonary artery comes from the left ventricle and is overflowed, pulmonary artery banding is useful for lowering the pulmonary blood flow.
  • In older children, bi-direction Glenn shunt which is the connection between superior vena cava to the pulmonary artery is planned for transferring the blood to the pulmonary system.
  • Fontan procedure is a conduit between the inferior vena cava and the pulmonary artery whether transfers the systemic venous blood to [[pulmonary circulation at the age of 2-3 years old.

Surgical management in d-TGA	Surgical management in Tricuspid Atresia
Life-saving balloon atrial septostomy in neonatal period	Blalock Taussig shunt
	*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
Atrial switch procedure	Pulmonary artery banding
Mustard procedure (baffle made of Dacron or pericardium) or Senning procedure(atrial flap) 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 Definition: Aorta arises from right ventricle and pulmonary artery arises from left ventricle	* 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
* Systemic to pulmonary artery shunt when pulmonary blood flow decreases In infants with TGA and VSD and PS 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 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-PA 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