Background Anomalous origin of the coronary artery from the pulmonary artery (ALCAPA) is a rare congenital disease. Surgical re-implantation of the left main coronary artery (LMCA) to the aorta is a definitive treatment with a good prognosis. Case Summary A 9-years old boy was admitted with a complaint of exertional chest pain and dyspnea. At 13 months of age, he was diagnosed to have ALCAPA as a workup of severe left ventricular systolic dysfunction and underwent coronary re-implantation of ALCAPA. Coronary angiogram displayed the high takeoff of re-implanted LMCA with significant ostial stenosis and echocardiogram showed significant supravalvular pulmonary stenosis (SVPS) with a peak gradient of 74 mmHg. After a multidisciplinary team discussion, he underwent percutaneous coronary intervention (PCI) with stenting to ostial LMCA. On follow-up, he was asymptomatic and a cardiac CT scan showed a patent stent in LMCA with an under-expanded area in the mid-segment. The proximal part of the LMCA stent was located very close to the stenotic segment of the main pulmonary artery making it a high risk for balloon angioplasty. The surgical intervention of SVPS is delayed to allow the somatic growth of the patient. Discussion Percutaneous coronary intervention in re-implanted LMCA is a feasible option. If stenosis of re-implanted LMCA is accompanied by SVPS, the latter can be best treated surgically and staged to decrease the operative risk. Our case also demonstrates the importance of long-term follow-up of postoperative complications of patients with ALCAPA.
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