Echocardiographic findings in Pompe's disease with left ventricular obstruction.

Two infants with Pompe's disease (type II glycogenosis) showing echocardiographic evidence of obstructive cardiomyopathy are described. On M-mode and two-dimensional (2-D) echocardiography there was a severe hypertrophy of the interventricular septum, free, and posterior left ventricular wall with midsystolic closure of the aortic valve. The combined echocardiographic and electrocardiographic findings are helpful in the clinical diagnosis of this severe disease.