Late myocardial infarct in pulmonary atresia and intact ventricular septum: A must-know complication!

Background Pulmonary atresia with intact ventricular septum is a rare congenital heart disease. Frequently associated coronary abnormalities impact on acute management but may also complicate long-term clinical course and worsen prognosis. Little is known about their clinical spectrum and management. Case summary An 8-year-old girl, born with pulmonary atresia and intact ventricular septum, who uneventfully achieved Fontan completion, presented with chest pain and high troponinemia. Electrocardiogram (ECG) showed significant acute repolarization anomalies ( Fig. 1 ). Cardiac magnetic resonance imaging (MRI) showed ischemic sequelae in the territory of the right coronary artery ( Fig. 2 ). Coronarography showed an abnormal diffuse collateral coronary network but no stenotic lesions of the main coronaries ( Fig. 3 ). Medical treatment was initiated with good recovery. Discussion Coronary abnormalities are frequently encountered in patients with atresia pulmonary with intact ventricular septum [1] . Abnormal sinusoids, fistulas but also diffuse modifications of the vessel walls are described who may significantly impact on neonatal management [2] , [3] . Our case report highlights the possibility of late coronary complications that invariably will impact on long-term prognosis. Close follow-up is warranted in all patients with pulmonary atresia and intact ventricular septum in order to detect ischemic events and treat them appropriately. The risk of ventricular arrhythmias and ventricular dysfunction may require the introduction of appropriate medical treatment or even more aggressive interventions such as implantable cardioverter-defibrillator or cardiac transplantation.