Echocardiographic sepctrum of the hypoplastic left heart syndrome: a clinicopathologic correlation in 19 newborns.

Ultrasonic studies were performed in 19 neonates with the hypoplastic left heart syndrome whose diagnosis was confirmed at angiography or autopsy, or both. The patients were classified in two echocardiographic groups: Group I, 10 infants whose ventricular septum could be recorded, and Group II, 9 infants whose septum could not be recorded. The findings in these groups were compared with those in 60 neonates without congenital heart disease also studied with ultrasound. Two additional neonates who presented with signs of shock were also studied. The diagnostic echocardiographic features of hypoplastic left heart syndrome were: (1)a left ventricular end-diastolic dimension of less than 9 mm; (2)an aortic root diameter of less than 6 mm; (3)a ratio of left ventricular end-diastolic to right ventricular end-diastolic dimension of less than 0.6; and (4)a mitral valve echo that is absent or greatly distorted and of small amplitude. These echocardiographic criteria differed significantly from findings in the normal group (P less than 0.01). Echocardiography proved valuable in neonates with shock. It is a safe, reliable technique that can be used to delineate the intracardiac anatomy in sick neonates with the hypoplastic left heart syndrome.