Anomalies of the coronary arteries in children.

BACKGROUND: Anomalies of the coronary arteries in children occur very rarely. Congenital coronary anomalies may be isolated, or they may accompany other congenital heart defects, such as Fallot's Syndrome, transposition of the great arteries, or pulmonary atresia. The most common cause of acquired abnormalities of the coronary vessels in children is Kawasaki Disease. The purpose of the present study is to analyze the clinical picture and diagnostic methodology for coronary anomalies in children.

MATERIAL AND METHODS: In 62,320 children a routine echocardiographic assessment was made of the ostium and the course of the initial segment of the coronary arteries. Anomalies of the coronary arteries were found in 29 children, ranging in age from 1 day to 12 years. In 25 of these cases the diagnosis was established on the basis of two-dimensional echocardiography with Doppler flow mapping, confirmed in 16 cases by aortography. In 3 children with Fallot's Syndrome and in 1 child with common arterial trunk, the coronary anomalies were found in angiocardiography.

RESULTS: Isolated abnormalities of the coronary vessels were found in 18 children, including 13 cases of anomalous left coronary artery from the pulmonary artery, 1 case of anomalous right coronary artery from the pulmonary artery, and four cases of coronary fistula. In 9 children anomalies of the coronary arteries accompanied other congenital heart defects: 4 children with Fallot's Syndrome were found to have left descending coronary artery arising from the right coronary artery; 1 child with a common arterial trunk was diagnosed with anomalous common origin of coronary arteries; 3 children with transposition of the great arteries were found to have coronary anomalies (including 2 cases of anomalous circumflex branch from the right coronary artery, and 1 case of intramural course of the left coronary artery), and one child with pulmonary atresia was found to have coronary fistulas. Of the 2 children with Kawasaki Disease, one was diagnosed with an aneurysm in the left coronary artery, and the other with dilatation of the coronary arteries. The infants with abnormal divergence of the left coronary artery showed clinical and electrocardiographic signs of myocardial ischemia, while the newborns with fistulas presented with symptoms of circulatory insufficiency. These children required emergency surgical treatment.

CONCLUSIONS: The clinical picture of children with anomalies of the coronary vessels may be heterogeneous, ranging from a lack of symptoms to very acute, even life-threatening symptoms. Infants with anomalous left coronary artery from the pulmonary artery and with hemodynamically significant fistulae require emergency surgical treatment. Echocardiographic examination enables the diagnosis of anomalies of the coronary arteries. In the majority of cases an aortography test is necessary before the patient is referred for surgery.