Abstract 4386: Single-Center Experience in Large Cohort of Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease over 15 years: Effect of New Therapies

TL;DRAbstract

Introduction: The aim of this study was to define the epidemiology, clinical parameters and 15-year follow-up of a large single-center cohort of patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD) and to evaluate the effect of emerging therapeutic strategies in this population. Methods: All patients diagnosed with PAH related to CHD between 1993 and 2008 were studied and clinical, echocardiographic, maximal and submaximal exercise as well as hemodynamic data were recorded. Results: A total of 60 pts aged 21 ± 14 (0.1–51) years at diagnosis were included in the study. Of these, 44 (73%) had unrestrictive mixing lesions and Eisenmenger syndrome and the rest non-mixing lesions, mainly repaired CHD. Of this population, 24 (40%) were <18yrs and the remainder adults at diagnosis at our institution. Seven (12%) pts in WHO Class II received no therapy, 45 (75%) pts in Class II-III monotherapy (34 bosentan with 1 discontinuation after corrective surg

Chat with Paper

AI Agents for this Paper