Pulmonary atresia with intact ventricular septum: is neonatal repair advisable?

Abstract

The optimal management of pulmonary atresia with an intact ventricularseptum in the neonate remains controversial. The introduction of balloonseptostomy and prostaglandin has significantly reduced early mortality butearly surgical intervention is necessary to obtain a more adequatepulmonary blood flow. Fourteen neonates with pulmonary atresia and anintact ventricular septum were admitted to the Wessex Cardiothoracic Unit,Southampton from 1979 to 1986. Thirteen patients underwent cardiaccatheterization. Cardiac catheterization data and right ventricularangiograms were reviewed retrospectively. Four patients with tripartiteventricles underwent total repair. The others received various palliativeoperations (valvotomy + modified Blalock- Taussig shunt or modifiedBlalock-Taussig shunt alone). Retrospective analysis of the angiogramsindicated that right ventricular morphology alone is not a satisfactorycriterion for surgical management. We have been able to demonstrate thatthere is a good correlation between the diameter of the tricuspid valve andthe diameter of the infundibulum and that successful neonatal repair ispossible when the tricuspid valve diameter is above 80% of the normal valuefor weight and when the tricuspid valve diameter to infundibular diameterratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartiteventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonaryvalvotomy with a modified Blalock-Taussig shunt remains the treatment ofchoice.