Surgical approach and rehabilitation in ARCAPA syndrome: report of a pediatric case
Keywords:
Arteria Pulmonar, Congenital Heart Disease, Cardiac Surgical Procedures, Cardiac Rehabilitation, Pulmonary ArteryAbstract
Introduction: anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA syndrome) is an extremely rare congenital malformation, with an estimated prevalence of 0.002% among all congenital heart defects. This condition is characterized by an anomalous origin of the right coronary artery from the pulmonary trunk, resulting in reverse coronary flow, risk of myocardial ischemia, and life-threatening complications, especially in the pediatric population.
Objective: to describe the case of a pediatric patient diagnosed with ARCAPA syndrome.
Case presentation: a 15-year-old male adolescent with no significant medical history presented with fatigue, anginal-like chest pain, and dyspnea on exertion. Physical examination revealed muffled heart sounds and asynchronous peripheral pulses. The electrocardiogram showed changes suggestive of ischemia, and cardiac magnetic resonance imaging confirmed the anomalous origin of the right coronary artery from the pulmonary artery with retrograde flow. Surgical correction was performed with an end-to-side anastomosis to the ascending aorta, followed by a cardiac rehabilitation program. The outcome was favorable, with complete restoration of cardiovascular function.
Conclusions: this condition is a potential cause of sudden death in young patients and should be included in the differential diagnosis of chest pain and exertional dyspnea. Accurate detection requires Doppler echocardiography and cardiac magnetic resonance imaging. Treatment with early surgical anastomosis restores coronary flow, complemented by cardiac rehabilitation. Multidisciplinary management involving cardiologists, surgeons, and rehabilitation specialists is essential for the therapeutic success of this complex congenital anomaly.
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Copyright (c) 2025 Miguel Enrique Barroso-Fontanals, Karla María Ascanio-Cruz , Carlos Roberto Urgelles-Oliva , Malena Laura Vázquez-Núñez
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