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Anomalous Origin of the Left Coronary Artery from Pulmonary Artery (ALCAPA)

Ronald Pachon, Claudio Bravo, Mark Niemiera

Congenital abnormalities, including anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), and lethal arrhythmias in a structurally normal heart explain about 5-10% of sudden cardiac death (SCD) cases. In this narrative review, the available literature pertaining to the rare congenital coronary artery malformation, ALCAPA, shall be comprehensively reviewed and summarized.

The majority of patients with ALCAPA present in early life with symptoms of ischemic heart disease often causing death. Alternatively, if sufficient collateral formation between the right and the left coronary arteries is present, these patients may be asymptomatic throughout their entire childhood. Since ALCAPA is rarely seen in pediatric patients and even less in adults, the diagnosis requires a high index of suspicion. The development of novel non-invasive high resolution imaging techniques such as echocardiography, multi detector computed tomography (MDCT) and cardiac MRI has allowed detailed visualization of the origin and course of the anomalous artery. Currently, surgical correction of the malformation is the optimal treatment option which can lead to significant improvement in myocardial ischemia. It is important to note, despite ALCAPA as a rare condition, this differential diagnosis should be always considered in an otherwise healthy young adult individual who presents with SCD, syncope, exercise intolerance or chest pain.