AORTIC-STENOSIS IN HYPOPLASTIC RIGHT HEART SYNDROME, ASSOCIATED WITH INTERSTITIAL DELETION OF CHROMOSOME-2

This report describes a rare case of moderate to severe valvular aorti c stenosis in the setting of severe tricuspid stenosis, pulmonary atre sia with intact interventricular septum and right ventricular dependen t coronary circulation. We discuss the clinical hemodynamic, echocardi ographic and angiocardiographic findings on this rare case and comment on accompanied malformations, with its possible association observed to the interstitial deletion of band q22 or q24 of the long arm of chr omosome 2. (C) 1997 Elsevier Science Ireland Ltd.