Clinical Characteristics of Congenital Atresia of the Left Main Coronary Artery in 12 Children.

Background: Left main coronary artery atresia (LMCAA) is an extremely rare abnormality and only <100 cases have been reported worldwide. We describe the clinical manifestations, imaging features, prognosis, and treatments of LMCAA who were admitted in our department, which aimed to improve the clinical diagnosis and treatments of LMCAA in children.

Methods: A retrospective study identified 12 patients diagnosed with congenital left coronary artery atresia at Pediatric Heart Center of Beijing Anzhen Hospital from June 2010 to June 2019. The clinical characteristics, imaging data, and treatment follow-up were analyzed.

Results: Among the 12 cases, 8 were boys and 4 were girls; the age of onset was 2 months to 2 years old (median age 7 months); the age of diagnosis was 7 months to 6 years old (median age 2 years and 11 months). At the initial diagnosis, there were 4 cases of respiratory tract infection with cardiac murmur, 3 cases of cardiac shadow enlargement, 1 case of recurrent syncope, 2 cases of feeding difficulty with cardiac enlargement, and 2 cases of simple cardiac murmur. In 12 cases of electrocardiogram examination, 7 cases showed pathological Q waves of lead I, AVL and v4-v6; in 12 cases of chest X-ray examination, 8 cases showed cardiac shadow enlargement; in 12 cases of our hospital's first cardiac ultrasound examination, 4 cases were definitely diagnosed, and 8 cases showed the possibility of left coronary artery abnormality; in 5 cases of cardiac coronary CT angiography examination, 2 cases were confirmed, 2 cases reported suspected left coronary artery abnormality, and 1 case did not report abnormality; All cases were definitely diagnosed in 8 cases of angiography. Follow-up was performed from 1 to 8 years; one case died suddenly, one case of syncope after activity was treated by oral medication, 3 cases received open coronary angioplasty and mitral valvuloplasty, recovered well after operation, the rest of the children were treated by oral medication, and the symptoms are stable at present.

Conclusions: Left main coronary artery atresia is difficult to diagnose and can result in heart failure early in life. Timely diagnosis and reasonable treatment are the keys to improve the prognosis.