Commentary: Another way to skin a rare and dangerous cat

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. 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. In many centers, patients with PA/IVS with RVDCC are directed toward cardiac transplantation rather than singleventricle palliation culminating in the Fontan circulation. Patients with RVDCC are at high risk of sudden death, particularly in the first few months of life, when pulmonary blood flow is provided by either a patent ductus arteriosus or a systemic to pulmonary shunt (SPS) with consequent diastolic hypotension. Often such patients demonstrate electrocardiographic evidence of myocardial ischemia, sometimes accompanied by elevations in serum levels of troponin or other markers of cardiomyocyte injury. 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. 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