Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum

Abstract

Characteristics of neonates with pulmonary atresia and intact ventricular septum predict type of definitive repair. A morphologically driven institutional protocol emphasizing both 2-ventricle and Fontan pathways might mitigate the negative effect of unfavorable morphology. In the current era, 85% of neonates are likely to reach a definitive surgical end point, with 2-ventricle repair achieved in an estimated 50%.

Indications for Heart Transplantation in Pediatric Heart Disease

“…The success of heart transplantation as a therapy for hypoplastic left heart syndrome led pediatric transplantation centers to apply heart transplantation as primary therapy in other conditions for which there was a poor natural history with standard surgical therapy. These conditions have included pulmonary atresia with intact septum and right ventricledependent coronary circulation 54,55 and complex heterotaxy syndromes 56,57 in which a functional single ventricle can be associated with anomalous pulmonary venous return and severe atrioventricular or semilunar valve disease.…”

Definition and Management of Segmental Pulmonary Hypertension

“…6 In extreme cases, the entire intrapericardial pulmonary arterial tree is absent and blood is supplied to the lungs by collateral arteries only. Some patients may be amenable to surgical repair 7, 8, 9. “Unifocalization” is a staged approach aimed at reconstructing the PAs and perfusing them by means of modified Blalock‐Taussig shunts with the ipsilateral subclavian arteries, or with other shunts as dictated by specific PA anatomy.…”

“…The lung supplied by a normal‐sized, unobstructed PA originating from the aorta is typically pressure and volume overloaded early in life and pulmonary vascular disease develops. Mortality is high in the first year of life (up to 70%) without timely repair 7. A small proportion of unrepaired patients do survive to adulthood and beyond, with segmental (eg, unilateral) PH.…”

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

“…53,54 Lower mortality in the current era may reflect (1) better selection of patients to decompress and attempt a biventricular repair, (2) the recognition of overcirculation and coronary steal in the initial palliation, and (3) the incorporation of transplantation as one of the therapeutic options. [55][56][57][58] With the overall survival having improved, the most practical approach may be to study the coronary circulation on diagnosis of those with a small tricuspid valve annulus, because it is now apparent that an RV-dependent coronary circulation typically exists in the hypoplastic RV unsuitable for biventricular repair. If an RV-dependent coronary circulation exists in an asymptomatic patient with normal left ventricular function and wall motion, then a reasonable option is to proceed with palliative surgery with a systemic-to-PA shunt with postoperative management that minimizes overcirculation, followed by early placement of a bidirectional cavopulmonary shunt and a Fontan procedure.…”