Aortic atresia with normal left ventricle and intact ventricular septum.
We report a case of aortic atresia with biventricular hypertrophy and death due to cardiac insufficiency (at 5 1/2 days of age). In the necropsy a normally sized left ventricle was observed, with endocardiac fibroelastosis, anomalous mitral arcade, intact interventricular septum and atresia of the aortic valve. The ascending aorta was moderately hypoplasic; there was a foramen ovale and persistent ductus arteriosus. We believe that this is the first case that has been reported of this anomaly occurring with a normal left ventricle and intact ventricular septum.
D001011:Aorta / Q000002:abnormalities / Q000473:pathology; D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology; D006332:Cardiomegaly / Q000473:pathology; D004374:Ductus Arteriosus, Patent / Q000150:complications / Q000473:pathology; D004695:Endocardial Fibroelastosis / Q000473:pathology; D006333:Heart Failure / Q000209:etiology; D006346:Heart Septum / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008943:Mitral Valve / Q000473:pathology