Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. Methods A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. Results Of the 37 patients identified, only 54% presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54% of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91%), collateral CA flow (85%), right CA dilation (81%), abnormal pulmonary artery flow (79%), mitral regurgitation (74%), left ventricular dysfunction (66%) and endocardial fibroelastosis (57%). Presenting echocardiograms had five of seven markers in 85% of patients. Left ventricular dysfunction was the most common marker in infants (89% vs 38%, P =.005); older children were more likely to have collateral formation visualized by color Doppler (100% vs 75%, P =.04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92% had normal left ventricular function, 3% had moderate or worse mitral regurgitation, and 17% had required reintervention. Conclusions Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.
Patel, S. G., Frommelt, M. A., Frommelt, P. C., Kutty, S., & Cramer, J. W. (2017). Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery. Journal of the American Society of Echocardiography, 30(9), 896–903. https://doi.org/10.1016/j.echo.2017.05.005