Characteristics and long-term outcome for congenital left main coronary artery atresia.

Abstract

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3years and 6years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2years old and 17years old, respectively. Two patients had no cardiac events without medication for more than 30years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.