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(Circulation. 1974;49:367.)
© 1974 American Heart Association, Inc.

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De Subitaneis Mortibus

IV. Coronary Vessels and Conduction System in Homocystinuria

THOMAS N. JAMES M.D.¹; NINA A. J. CARSON M.D.¹; PETER FROGGATT M.D., PH.D.¹

¹ From the Department of Medicine, University of Alabama Medical Center and the Faculty of Medicine, Queen's University of Belfast.

Conspicuous abnormalities of the coronary arteries and veins and of the sinus node, atrioventricular (A-V) node and His bundle are described in the heart of a boy dying with congenital homocystinuria. The unproven possibility of transient disturbances of cardiac rhythm or conduction is discussed relative to the pathogenesis of the bouts of unconsciousness with and without convulsions characteristic of the disease. Factual information on the stability or instability of electrical activity of the heart in such patients is particularly needed because of the possibility of treatable aspects of an otherwise seemingly inexorable process.

Key Words: Platelet dyscrasia • Coronary venous pathology • Sinus node • A-V node • Sudden death

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