ANOMALOUS ORIGIN OF CORONARY-ARTERIES AND RISK OF SUDDEN-DEATH - A STUDY BASED ON AN AUTOPSY POPULATION OF CONGENITAL HEART-DISEASE

Coronary arteries anomalies may be part of complex congenital malforma tions of the heart or be an isolated defect. In our anatomic collectio n of congenital heart disease, an isolated anomalous origin of coronar y arteries was observed in 27 of 1,200 specimens (2.2%): left coronary artery from pulmonary trunk in five, origin from the wrong aortic sin us in 12 (both right and left coronary artery from the right sinus in four and from the left sinus in seven, left coronary artery from the p osterior sinus in one), left circumflex branch from right aortic sinus or from very proximal right coronary artery in three, high takeoff of right coronary artery in three, stenosis of the coronary ostia attrib utable to valvelike ridge in four. In 16 (59%) patients (12 males and 4 females, age ranging from 2 months to 53 years; median, 14), the fin al outcome was sudden death; it occurred in all cases of left coronary artery origin from right aortic sinus, in 43% of right coronary arter y origin from left aortic sinus, and in 40% of the left coronary arter y from the pulmonary trunk. Sudden death was precipitated by effort in eight (50%) and was the first manifestation of the disease in eight ( 50%); previous symptoms consisted of recurrent syncope in four, palpit ations in three, and chest pain in one. Five patients who died suddenl y during effort were athletes. In conclusion, (1) more than half of ou r postmortem cases with anomalous origin of coronary arteries died sud denly, (2) all but two patients with sudden death had anomalous corona ry artery origin from the aorta itself, (3) the fatal event was freque ntly precipitated by effort, (4) palpitations, syncope, and ventricula r arrhythmias were the only prodromic symptoms and signs. Recognition during life of these coronary anomalies, by the use of noninvasive pro cedures, is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated. Copyright (C) 1998 by W.B . Saunders Company.