Acute Coronary Syndrome in Young Adults with Hypoplastic Left Heart Syndrome: Treatment and Outcome

All articles of this category (opens in new window) Background: Thromboembolic events are a common complication in patients with total cavopulmonary connection, whereas acute coronary syndrome is rarely observed in this population. In the past, case reports of myocardial infarction in patients with hypoplastic left heart syndrome (HLHS) due to thrombus formation within the native aortic root have been published. Methods: We report the case of a 22-year-old woman with HLHS and Fontan circulation who presented with an acute onset of typical angina pectoris symptoms. The electrocardiography (ECG) showed new ST elevations in the precordial leads, indicative of an acute myocardial infarction. Coronary angiography revealed thrombotic occlusion of the proximal right coronary artery (RCA). Percutaneous coronary revascularization was performed by thrombus aspiration, RCA angioplasty, and implantation of a drug-eluting stent into the RCA. Despite a good revascularization result, severely impaired function of the systemic right ventricle (RV) did not recover, and the patient required extracorporeal life support (ECLS) on day 1 after RCA revascularization. As the RV function did not recover over the following days and the patient developed multiorgan failure, heart transplantation was not an option. Eventually, the patient died while on ECLS due to multiorgan failure. Conclusion: This case highlights that acute coronary syndrome can also represent a thromboembolic complication in HLHS patients after the Norwood and Fontan procedures, due to thrombus formation within the aortic root with subsequent embolization into the RCA, and should be considered in the differential diagnosis when appropriate symptoms occur. Oral anticoagulation might, therefore, be considered in HLHS patients at risk for thrombosis within the native aortic root. Risk factors, however, still have to be defined. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany