Atrioventricular septal defect with intact atrial and ventricular septal structures.
A heart with an atrioventricular septal defect is characterized by absence of the atrioventricular muscular and membranous septa, a common atrioventricular junction, an unwedged position of the aortic valve annulus and disproportionate inlet--outlet dimensions of the ventricular septum. The clinical and post-mortem findings are described of a case which had intact atrial and ventricular septa but had all the other anatomical hallmarks of atrioventricular septal defect. The problems in clinical diagnosis may be overcome by cross-sectional echocardiography.
D006332:Cardiomegaly / Q000473:pathology; D004452:Echocardiography / Q000379:methods; D004694:Endocardial Cushion Defects / Q000473:pathology*; D005260:Female; D006325:Heart Atria / Q000033:anatomy & histology; D006343:Heart Septal Defects / Q000473:pathology*; D006346:Heart Septum / Q000033:anatomy & histology; D006352:Heart Ventricles / Q000033:anatomy & histology; D006801:Humans; D007223:Infant