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.

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.

Coronary abnormalities are frequently encountered in patients with atresia pulmonary with intact ventricular septum [1Calder A.L., Sage M.D. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum Am J Cardiol 1987 ;  59 (5) : 436-442 [cross-ref]

Cliquez ici pour aller à la section Références]. Abnormal sinusoids, fistulas but also diffuse modifications of the vessel walls are described who may significantly impact on neonatal management [2L’Ecuyer T.J., Poulik J.M., Vincent J.A. Myocardial infarction due to coronary abnormalities in pulmonary atresia with intact ventricular septum Pediatric Cardiol 2001 ;  2 (1) : 68-70 [cross-ref]

Cliquez ici pour aller à la section Références, 3Elias P., Poh C.L., du Plessis K., Zannino D., Rice K., Radford D.J., et al. Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischemia and death Eur J Cardiothorac Surg 2018 ;  53 (6) : 1230-1236 [cross-ref]

Cliquez ici pour aller à la section Références]. 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.