Long-term management of a child with aortopulmonary septal defect and Eisenmenger syndrome: a 6-year follow-up case study

We report the case of a boy diagnosed at age 9 with a large aortopulmonary septal defect and Eisenmenger syndrome, highlighting the challenges of late diagnosis and management of fixed pulmonary hypertension. Over the course of six years (2018–2024), the patient underwent several follow-ups, including invasive hemodynamic assessments and echocardiography, revealing progressive pulmonary vascular disease. Despite the administration of medical therapy with sildenafil, bosentan, and antiplatelets, the patient’s condition remained unfavorable due to fixed pulmonary vascular obstructive disease. This case underscores the importance of early diagnosis and the complexities of managing advanced pulmonary hypertension in congenital heart defects.