Commentary: Another way to skin a rare and dangerous cat.

Robert D B Jaquiss

doi:10.1016/j.xjtc.2021.02.033

Abstract

In patients born with pulmonary valve atresia with intact ventricular septum (PA/IVS), right heart structures may be so hypoplastic that biventricular circulation cannot be achieved.1 In the most unfavorable manifestation of PA/IVS, the epicardial coronary arteries are stenotic (or proximally atretic) such that a significant amount coronary arterial flow is provided by the hypoplastic and hypertensive right ventricle via sinusoidal connections to the coronary arteries. This condition is termed “right ventricular dependent coronary circulation” (RVDCC), the most extreme form of which is bilateral coronary ostial atresia.

Highlights

In patients born with pulmonary valve atresia with intact ventricular septum (PA/IVS), right heart structures may be so hypoplastic that biventricular circulation cannot be achieved.[1]
One approach to address the coronary insufficiency in right ventricular dependent coronary circulation” (RVDCC) is to provide “antegrade” blood flow to the right ventricular cavity by creation of an aorto-right ventricle shunt (ARVS)
In pulmonary atresia/intact septum with bilateral coronary ostial atresia, antegrade coronary blood flow may be optimized by an aorto-right ventricular shunt, to the free wall or tricuspid annulus

Summary

Introduction

In patients born with pulmonary valve atresia with intact ventricular septum (PA/IVS), right heart structures may be so hypoplastic that biventricular circulation cannot be achieved.[1]. One approach to address the coronary insufficiency in RVDCC is to provide “antegrade” blood flow to the right ventricular cavity by creation of an aorto-right ventricle shunt (ARVS). This was initially described in an older child at the time of Fontan completion.[4] Later, a small series was reported with placement of ARVS in 5 children at varying ages, the youngest of whom was 6 weeks of age and

Results

Conclusion