Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA): a Systematic Review and Historical Perspective

Abstract

To provide an updated literature review for the diagnosis, treatment, and outcomes of anomalous left coronary artery from the pulmonary artery (ALCAPA). The diagnosis of ALCAPA has shifted away from coronary angiography to noninvasive imaging modalities. Newer imaging techniques such as speckle tracking echocardiography to evaluate myocardial strain demonstrate subclinical myocardial dysfunction years after restoration of a dual coronary system. The diagnosis of ALCAPA is primarily with echocardiography and computed tomography coronary angiography. Coronary reimplantation is the preferred surgical technique. Long-term outcomes are excellent, greater than 97% survival in the modern era, and most patients will have resolution of their systolic dysfunction, with the most common indication for reintervention being mitral valve regurgitation. Some patients with successful reestablishment of a dual coronary arterial system have subclinical myocardial dysfunction, detected by myocardial strain echocardiography, in the setting of normal systolic and diastolic parameters. Further research is needed to determine the impact and long-term outcomes of subclinical dysfunction on long-term survivors.