Anomalous Left Coronary Artery from the Pulmonary Artery: Images Studies of Echocardiographic, Heart CT and Angiography

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare but serious congenital coronary arterial anomaly. Congestive heart failure, myocardial ischemia &even sudden cardiac death may occur in early infancy. Early diagnosis is usually difficulty before the occurrence of clinical symptoms. Surgical correction is usually necessary as soon as possible to avoid the long-term sequel of myocardial ischemia & significant mitral regurgitation. A 3 months old female infant was sent to pediatric clinic due to shortness of breath for two days . Physical examination revealed mild subcostal retraction, a grade II/VI systolic murmur over left lower sternal boarder, hepatomegaly with 3 cm below right costal margin. The peripheral arterial pulsations were normal and without no cyanosis, Chest X-ray film showed moderate cardiomegaly (Figure 1) with normal pulmonary vascularity. She was referred to pediatric cardiologic clinic and echocardiography revealed a single right main coronary artery with diameter of 2.2mm (Figure 2) and moderate to severe mitral valve regurgitation (Figure 3) . The ejection fraction of left ventricle was 12.5%. Emergency heart CT showed only the single right coronary artery (Figure 4). Cardiac catheterization and angiography (Figure 5) confirmed the diagnosis of anomalous origin of left coronary artery from the main pulmonary artery (ALCAPA). Then she received the surgical total correction with re-implantation of the left coronary artery from main pulmonary artery to ascending aorta. Post-operative recovery was not unevenly. She was under anti-congestive therapy and follow up at pediatric cardiologic clinic regularly. The follow-up echocardiography revealed good anastomosis of left coronary artery with aorta, moderate mitral regurgitation and nearly normal left ventricular function with ejection fraction of 58.97 %. Although ALCAPA is a very rare congenital coronary artery anomaly, but it is usually presented with sudden onset of congestive heart failure, myocardial ischemia, even cardiac death in infancy when the high pulmonary arterial pressure dropped down as normal sequence. Echocardiography can usually identify only the right coronary artery from aorta, mitral regurgitation and dilatation of left ventricle and atrium. Other images studies such as heart CT and angiography are the accurate diagnostic tool. Post-operative follow up with echocardiography is necessary to assess the anastomosis of re-implanted left coronary artery, the severity of mitral regurgitation and ventricular function.