Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery (ARCAPA) in Adults: Analysis of 59 Clinical Cases.

Background

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an extremely rare and potentially fatal congenital heart defect, accounting for approximately 0.003% of all coronary artery anomalies.

Methods

A literature search was performed in the PubMed and eLibrary databases using the keywords "ARCAPA" and "anomalous origin of the right coronary artery from the pulmonary artery" for the period from 2005 to 2025. A total of 158 papers were screened, from which 57 articles reporting 59 clinical cases in adult patients were selected.

Results

The median age was 55 years [18-80]. Males accounted for 54.2% (32/59). ARCAPA was an incidental finding during evaluation for another condition in 35.9% (21/59) of cases. Clinical presentation: retrosternal pain-47.5% (28/59), dyspnea-44.1% (26/59), and reduced exercise tolerance-35.9% (21/59). A completely asymptomatic course was observed in 22.0% (13/59) of patients. In rare cases, the initial presenting manifestations included atrial fibrillation, cardiac arrest during a marathon, and worsening dyspnea during pregnancy. The most commonly used diagnostic modalities were computed tomography angiography-in 67.8% (40/59) of cases-and coronary angiography-in 49.2% (29/59) of cases. Cardiac catheterization confirmed the diagnosis in only 10.2% (6/59) of patients. Surgical correction was performed in 54.2% (32/59) of patients; the most frequently used technique was reimplantation of the right coronary artery into the ascending aorta-in 42.4% (25/59) of cases. One long-term complication was recorded-right coronary artery thrombosis 17 years after surgery-and one case of sudden cardiac death occurred in an unoperated patient. Conservative management was chosen in 40.7% (24/59) of patients, mainly due to an asymptomatic course, high surgical risk, or patient refusal.

Conclusions

ARCAPA is a rare anomaly for which surgical treatment appears to be the preferred approach in symptomatic patients or those with documented ischemia, while conservative management may be acceptable in selected asymptomatic patients. Conservative management is acceptable in truly asymptomatic patients, but regular follow-up is recommended.