Daniela Milanova-Ilieva, Kostadin Ketev, Ivanka Karavelikova, Lyubov Chochkova-Bukova, Elisaveta Levunlieva, Anna Kaneva-Nencheva
St George University Hospital. Medical University of Plovdiv, Plovdiv. National Heart Hospital. National Cardiology Hospital.
Bulgaria
Folia Medica
Folia Med 2026; 68:
DOI: 10.3897/folmed.68.e149000
Abstract
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes