Congenital Septal Dysplasia of the Heart Causing Sudden Death

AORTIC STENOSIS has long been recognized as a cause of sudden death. The inevitably bad prognosis in obstructive disease of the left ventricle has led to intensive diagnostic study and courageous surgical assault, with impressive results in recent years. As a consequence of new interest stimulated by successful surgical approaches, variant forms of obstruction to the left ventricular outflow tract are being reported. Brock¹ has noted obstruction to the left ventricle in cases of hypertrophy, which he attributes to hypertensive disease. Bercu and associates² have described pseudoaortic stenosis in the presence of symmetrical hypertrophy without hypertension. An intriguing concept was offered by Teare³ in 1958, in which he suggested that hypertrophy of the ventricular septum might compromise the outflow tract of the left ventricle and cause a clinical syndrome simulating that of aortic stenosis and resulting in sudden death. A recent clinical experience corroborates the findings