Ectasia of the coronary arteries.

Abstract

Various abnormalities of the coronary arteries have been described in tetralogy of Fallot. This communication reports ectasia of the coronary arteries in 3 patients with tetralogy of Fallot. The anomaly has not been described previously. The first case was of a 24-year-old male with tetralogy of Fallot and pronounced cyanosis. The hematocrit was 79 per cent. On preoperative right ventricular angiocardiography findings typical for the tetralogy of Fallot were present, and, in addition, the coronary arteries were enormously dilated. An aortogram (Fig. 1) showed these enormously dilated coronary arteries to better advantage. They had a normal origin from the aorta. The dilatation and tortuosity of the right coronary artery and its branches were particularly marked. There was no abnormal communication between the coronary arteries, or between them or any of the heart chambers or other vessels. At total correction of the tetralogy of Fallot (Prof. V. O. Björk), the tortuous and 10–12 mm wide arteries were again observed. They were located mainly on the surface of the heart, could be pushed aside, and did not prevent the surgical procedure. The total correction was successful, but failure of the left ventricle developed, and the patient died in the immediate postoperative period. At autopsy the angiographic and surgical findings were confirmed. Microscopic examination of the walls of the dilated coronary arteries revealed no abnormalities. The other 2 cases of this report were in children, two and three years of age, with tetralogy of Fallot. Wide coronary arteries and a tortuous right coronary artery were demonstrated (Fig. 2), but the changes were not nearly as marked as in the adult. The cause of this dilatation of the coronary arteries remains obscure. These 3 patients were all very cyanotic, but in our material of 71 cases of tetralogy of Fallot there are many others with the same degree of cyanosis but without this remarkable dilatation of the coronary arteries. There was no obvious correlation between this coronary artery anomaly and any other anatomical or pathophysiological findings in these 3 cases. While apparently the abnormality exists in children and may become more pronounced with increasing age, it was no more frequent in adults with tetralogy of Fallot than in children. A correct preoperative angiocardiographic diagnosis of this rare anomaly is important. It should not be confused with other abnormalities of the coronary arteries which may contraindicate or make surgical total correction of the tetralogy of Fallot difficult. Summary A rare and previously undescribed anomaly consisting of marked dilatation and tortuosity of the coronary arteries was observed in one adult and two children with tetralogy of Fallot.