The surgical management of tetralogy of Fallot (TF) may be complicated by anomalies in the course and distribution of coronary arteries. Selective coronary angiography was performed in 119 cases of TF in order to prevent injury of aberrant vessels. In 11 patients, anomalies in the origin of coronary branches were revealed (five patients with a single coronary ostium, five with the anterior descending coronary artery arising from the right coronary artery or right sinus of Valsalva, and one with the circumflex artery arising from the right coronary artery). Other findings include anastomoses between coronary and bronchial arteries, small fistulas between coronary arteries and the pulmonary artery or right atrium, and hypoplasia of the coronary tree. The onset of left anterior hemiblock after total correction of TF could possibly be explained, in cases with a dominant left pattern, by injury to the coronary system after closure of the interventricular septal defect, rather than by injury to the conduction system.

Numerous studies have pointed out the frequent association of tetralogy of Fallot (TF) with other cardiovascular defects and coronary tree anomalies. We found cardiac defects in 181 (68%) out of 265 patients with TF investigated by catheterization and selective coronary angiography. These anomalies were isolated in 88 cases (49%) and associated with others in 93 patients. In the case of an isolated anomaly associated with TF, the coronary tree was involved in 37.5% and the cardiovascular system in the remaining 62.5%; in the case of two anomalies, the coronary system was involved in 66% of the patients and the cardiovascular apparatus in 34%; in the case of three or more anomalies, the coronary arteries were involved in 71% and the cardiovascular system in 29%. Anomalies in the course and/or distribution of coronary arteries were present in 96 patients (36%): 10 had a single coronary ostium, 13 a left anterior descending artery arising from the right coronary artery, one a circumflex artery arising from the right coronary artery. Small fistulas between coronary arteries and the pulmonary artery were found in 20 cases; anastomoses between coronary and bronchial arteries or right atrium in 42. In 39 patients we observed a large conus artery or large anterior ventricular branches crossing the right ventricle. A right aortic arch was found in 56 patients (21%), a stenosis of the trunk and/or the peripheral pulmonary artery in 35 (13%) and pulmonary artery atresia in five. Four patients showed a complete atrioventricular canal, three an atrial septal defect (primum type) with cleft of the mitral valve, 61 (23%) an atrial septal defect (ostium secundum). Eleven patients had anomalies of the systemic venous return, 26 (10%) a patent ductus arteriosus. Four patients had valvular abnormalities. In our series, a large proportion of cardiac defects associated with TF consists of anomalies of coronary arteries. Our data confirm the usefulness of performing preoperatively routine coronary angiography in patients with complex congenital heart disease.

Congenitally corrected transposition of the great arteries is an unusual cardiac malformation with discordant atrioventricular and ventriculoarterial alignments. Because knowledge of the coronary artery anatomy is a prerequisite for successful repair of this cardiac anomaly, selective coronary arteriography was performed in 13 children (4 male and 9 female; age range 18 months to 16 years) and 1 adult (aged 59 years) with congenitally corrected transposition of the great arteries and associated intracardiac defects. The typical coronary distribution of corrected transposition (that is, coronary artery-ventricular concordance) was found in 11 patients. In one patient, a single coronary ostium was observed; the right sinus of Valsalva gave rise to a short common branch that divided into three arteries: a left circumflex artery going to the right, a well developed left anterior descending artery running into the anterior interventricular groove and a third vessel that continued on the normal course of the right coronary artery directed posteriorly. In one patient, the left circumflex artery was particularly small. In another patient, with severe hypoplasia of the left anterior descending coronary artery, the anterior ventricular wall of the heart was supplied by three small branches that ended a short distance from their origins. The adult patient had a large anterior ventricular branch arising from the morphologic left coronary ventricular as well as a large acute marginal branch, with a wide distribution, from the morphologic right coronary artery. Presurgical coronary angiographic documentation is helpful because, in congenitally corrected transposition as well as in complex congenital heart disease, coronary anomalies (in origin, course and distribution) are occasionally present and knowledge of their presence can help determine the most appropriate surgical approach.

A child with a large ventricular aneurysm was completely free of symptoms when admitted to the hospital; a chest-x-ray previously performed because of acute pneumonia had evidenced a bulge of the left border of the heart. The diagnosis of left ventricular aneurysm was verified by left ventricular angiography. Selective coronary angiography and angiocardiography of the right heart were also performed.