Abstract
The constellation of semilunar valve atresia, important ipsilateral ventricular hypoplasia, intact ventricular septum, and associated anomalies of the coronary arterial tree is rare but well described. Although it occurs with aortic valve atresia, it is more commonly seen with pulmonary valve atresia and is especially difficult to manage if the coronary arteries are obstructed proximally to such an extent as to cause myocardial ischemia. In such cases, the term right ventricular–dependent coronary circulation (RVDCC) is used.
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