Retrospective Review on Anomalous Left Coronary Artery from Pulmonary Artery

Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare but important congenital coronary anomaly. Patients with ALCAPA typically present with paroxysms of crying, pallor, diaphoresis, agitation and heart failure in infancy due to myocardial ischaemia. Clinical course thus very much depends on the presence and development of collateral circulation. We report the clinical presentation and method of diagnosis of ALCAPA in Chinese children in Hong Kong. We also study their long term outcomes after surgery and compare local data with overseas results. Early diagnosis and correction are important to avoid development of or progression of mitral regurgitation, which appeared to be associated with lower chance of requiring mitral valve annuloplasty/replacement. The majority of patients in Hong Kong presents as infantile form with heart failure, mitral regurgitation and dilated cardiomyopathy. Typical ECG changes are present in 5 cases (55.6%). Echocardiography confirms the diagnosis in 3 cases (33.3%). The remaining patients require cardiac catheterization to confirm the diagnosis. Early diagnosis therefore relies on a high index of suspicion. The diagnosis of ALCAPA should be considered as differential diagnosis of mitral regurgitation, endocardial fibroelastosis and dilated cardiomyopathy. Cardiac catheterization is warranted in doubtful cases. Long-term survival and outcome after surgical treatment appear to be favourable with good functional status. Residual lesions occur in 4 (44.4%) patients and re-operation on the mitral valve is required in 1 (11.1%) patient. Our local results are comparable to overseas reports.