ALCAPA: navigating the complexities of a rare congenital heart defect—a narrative review

This narrative review aimed to synthesize the current evidence on anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), with particular emphasis on age-dependent presentation, multimodality diagnosis, age-tailored surgical decision-making, long-term postoperative issues, and realistic future directions for developmental and genomic research. PubMed, Embase, and the Web of Science Core Collection were searched for English-language publications from January 1933 to August 2025 using combinations of “ALCAPA,” “anomalous left coronary artery from the pulmonary artery,” “Bland-White-Garland,” “diagnosis,” “surgery,” “mitral regurgitation,” “outcome,” “embryology,” and “genetics.” Original studies, reviews, guideline documents, and illustrative case series relevant to epidemiology, imaging, operative management, and follow-up were included; duplicate publications, non-human mechanistic reports without translational relevance, and reports lacking sufficient clinical detail were excluded. Evidence was synthesized narratively because of marked heterogeneity in patient age, anatomy, operative strategy, and outcome reporting. ALCAPA remains a rare but potentially lethal coronary anomaly. Echocardiography is the preferred first-line screening tool, whereas computed tomography angiography and cardiac magnetic resonance provide higher anatomic and functional resolution for confirmation, operative planning, and postoperative surveillance. Surgical restoration of a 2-coronary-artery system is recommended irrespective of age once the diagnosis is established, but the preferred technique differs according to coronary anatomy, patient age, and tissue mobility. Direct coronary reimplantation is generally favored when feasible; Takeuchi repair and coronary artery bypass grafting remain selective alternatives with distinct late complications. In contrast, the genomic literature is still sparse and currently provides hypotheses rather than clinically actionable biomarkers.