Beyond the Usual Suspects: Unmasking ALCAPA in Pediatric Cardiomyopathy – Case Report

Introduction: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially lethal congenital heart disease. Delayed diagnosis can result in irreversible myocardial dysfunction. Although the classic presentation occurs in infants, atypical forms may appear in older children, making this entity a diagnostic challenge. Case Presentation: We report the case of a 9-year-old male with a history of dilated cardiomyopathy and uncontrolled asthma, admitted for an asthma exacerbation. Echocardiography revealed left ventricular dysfunction, severe mitral regurgitation, and pulmonary hypertension. Additional studies, including cardiac magnetic resonance imaging and catheterization, confirmed the diagnosis of ALCAPA. Notably, echocardiographic hallmarks such as collateral circulation and retrograde filling of the left coronary artery were absent, rendering this case particularly unusual. The patient underwent coronary reimplantation and mitral valvuloplasty, achieving a satisfactory postoperative outcome. Discussion ALCAPA can mimic dilated cardiomyopathy and should be suspected in patients with left ventricular dysfunction, mitral regurgitation, and heart failure. Echocardiographic findings such as segmental wall motion abnormalities, papillary muscle echogenicity, and mitral regurgitation may alert clinicians to this diagnosis. While echocardiography is part of the initial assessment, definitive diagnosis usually requires catheterization or coronary CT angiography. Conclusion This case underscores the importance of early recognition and surgical intervention in ALCAPA, particularly in atypical presentations. Clinical suspicion, targeted imaging, and multidisciplinary management are critical to improve prognosis in pediatric patients with complex congenital heart disease.