SUDDEN CARDIAC DEATH IN A CHILD, CAUSED BY AN ANOMALOUS ORIGIN OF THELEFT CORONARY-ARTERY FROM THE RIGHT SINUS OF VALSALVA

A seven-year-old girl suddenly collapsed during a sports session at sc hool. An orthostatic syndrome was assumed. In the next two-and-a-half years she experienced four further attacks of weakness with vertigo an d vomiting. Paediatric cardiological examination found her to be below the 3rd percentile for height, but the other physical findings and a thoracic radiogram were according to her age. The resting ECG showed i ncreased T waves in V5 and V6, interpreted as signs of vagotonia. Echo cardiography was normal, the results of the Schellong test suggested a bnormal hypotonic circulatory regulation. Five months later the girl d ied suddenly after physical exertion. The post-mortem revealed anomalo us origin of the left coronary artery (LCA) with a slit-like ostium fr om the right sinus of Valsalva. The very narrow initial part of the LC A passed tangentially through the wall of the aortic root and then too k a leftward course between the aortic root and the pulmonary trunk. - It is assumed that the sudden cardiac death resulted from compression of the intramural segment of the LCA.