Aortic-mitral valve discontinuity has previously been described in double outlet right ventricle, endocardial cushion defect, single ventricle, tetralogy of Fallot, and prolapse of the mitral valve. We are reporting two additional examples of aortic-mitral valve discontinuity including 15 cases of gross left ventricular dilation and a case of acute pneumococcal bacterial endocarditis with a la...

AMONG the congenital malformations in t& which an abnormal relationship exists between the aorta and pulmonary trunk is that in which both of these vessels arise from the right ventricle. The only outlet for the left ventricle is a ventricular septal defect. Right ventricular infundibular stenosis may or may not be associated. These cases apparently are extremely rare,1-3 and only a few have be...

AMONG the congenital malformations in t& which an abnormal relationship exists between the aorta and pulmonary trunk is that in which both of these vessels arise from the right ventricle. The only outlet for the left ventricle is a ventricular septal defect. Right ventricular infundibular stenosis may or may not be associated. These cases apparently are extremely rare,1-3 and only a few have be...

The benefits of a baffle fenestration in essentially high-risk Fontan patients have been demonstrated. Described here is the use of a new fenestration between the left atrial appendage and the left superior vena cava after Kawashima operation in a patient with a double-outlet right ventricle with hypoplastic left ventricle, left atrial isomerism, bilateral superior vena cavae with no bridging v...

Aortic-mitral valve discontinuity has previously been described in double outlet right ventricle, endocardial cushion defect, single ventricle, tetralogy of Fallot, and prolapse of the mitral valve. We are reporting two additional examples of aortic-mitral valve discontinuity including 15 cases of gross left ventricular dilatation and a case of acute pneumococcal bacterial endocarditis with a l...

Twenty-six patients with atrioventricular discordance and a variety of ventriculo-arterial connections have had appropriate intracardiac repairs. Seven of 26 patients (26.9%) died early postoperatively, but only two (11.1%) of 18 patients operated upon since May 1972 have died. Ten patients (40%) developed permanent complete heart block. Two patients died late postoperatively; 16 (84.1% of thos...

A double outlet right ventricle was diagnosed in a 10 week old infant, in whom the ventricular septum was intact at the time of presentation. A large aneurysm of a membranous septal remnant was present, and this appears to be the first recorded instance of closure of a malalignment ventricular septal defect by such a mechanism.

Six patients with early systolic closure of the aortic valve are described with mitral regurgitation, double outlet right ventricle, left ventricular diverticulum, congestive cardiomyopathy, Eisenmenger ventricular septal defect, and aortic regurgitation with an aneurysmal ascending aorta, respectively. None had evidence of subaortic stenosis. Early sytolic closure of the aortic valve is thus n...