Unrepaired Truncus Arteriosus Type 1 With Eisenmenger Syndrome and Recurrent Embolic Strokes: An Adult Case Report

BACKGROUND In adults, non-corrected truncus arteriosus type 1 with Eisenmenger syndrome is a rare congenital heart defect characterized by a single large artery from the heart that directs blood flow to the lungs, leading to irreversible pulmonary hypertension and a reversal of blood flow. This report describes the case of a 44-year-old woman with congenital Eisenmenger syndrome and unrepaired truncus arteriosus type 1 presenting with recurrent thromboembolic cerebral infarcts. CASE REPORT A 44-year-old woman presented with a 2-month history of recurrent neurological deficits, including aphasia, right-sided weakness, and vertigo. Magnetic resonance imaging (MRI) of the brain confirmed acute embolic infarcts, while computed tomography (CT) angiography ruled out large-vessel occlusion. Cardiac evaluation revealed a 15-mm perimembranous ventricular septal defect with a bidirectional shunt and a severely calcified truncal valve with moderate regurgitation. Imaging confirmed truncus arteriosus type 1, with the pulmonary arteries originating from the ascending aorta. The right ventricular systolic pressure was estimated at 114 mmHg, and baseline oxygen saturation (SpO₂) was 70-80%. Due to irreversible pulmonary vascular disease, she was not a candidate for surgical repair. A conservative management strategy was adopted, including dual antiplatelet therapy, sildenafil, macitentan, bisoprolol, and oxygen therapy. At the 6-month follow-up, the patient remained neurologically stable with her SpO₂ improved to 85-88%. CONCLUSIONS This case highlights the exceptional natural course of uncorrected truncus arteriosus in adulthood. It underscores that for patients with Eisenmenger physiology where surgical repair is not feasible, a multidisciplinary approach focusing on tailored antithrombotic and pulmonary vasodilator therapies is essential for stroke prevention and clinical stability.