When Congenital Meets Coronary: Chest Pain in a Patient with PA-IVS and Fontan Circulation

All articles of this category (opens in new window) Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is present in 0.3% of all congenital heart defects and can manifest itself in very different ways. The degree of right ventricular hypoplasia limits the possibility of establishing biventricular circulation. Methods: A 23-year-old female patient was admitted to the chest pain unit with angina-like chest pain. The patient, who suffered from PA-IVS, hypoplasia of the right ventricle, a missing right ventricular outflow tract (RVOT), and a missing pars trabeculate, had undergone palliative surgery during her childhood, which resulted in a total cavopulmonary connection (TCPC) with intracardiac tunneling and atrial fenestration. Follow-up examinations in pediatric cardiology were discontinued when she reached adulthood. The initial diagnosis revealed changes suggestive of ischemia in the posterolateral leads of the 12-lead ECG. Laboratory examinations showed elevated troponin levels, which increased dynamically from 137 to 628 pg/mL within 2 hours. Echocardiography showed hypokinesia of the apical segment with preserved overall systolic function. INR values were subtherapeutic. Coronary angiography confirmed a thrombotic occlusion of the left anterior descending artery (LAD). Balloon-only angioplasty did not achieve complete recanalization, and stent implantation was successfully performed (2.0 mm × 15 mm). Another cardiac catheterization showed pervious Fontan circulation (Fontan pressure 9 mm Hg, transpulmonary gradient 2–3 mm Hg), sub systemic pressure in the right ventricle, presence of severe tricuspid regurgitation, and any retrograde perfused coronary fistulas and right ventricular coronary anomalies were ruled out. Results: Several potential mechanisms of coronary ischemia must be considered in patients with congenital heart disease: congenital coronary anomalies and coronary fistulas caused by pressure overload of the right ventricle can lead to ischemic complications and right ventricular coronary circulation (RVDCC), which carries a risk of sudden cardiac death. In the present case, paradoxical embolism due to persistent fenestration of the intracardiac tunnel appears to be the most likely cause of myocardial infarction. Conclusion: This case highlights the need for appropriate interdisciplinary care for adults with congenital heart defects. Close interdisciplinary collaboration between cardiologists and disease-specific treatment is essential to ensure appropriate therapy and early detection of possible complications. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany