Severe pulmonary arterial hypertension associated with congenital cardiac shunts: evolution under specific treatment

Objective: The aim of this study was to compare the effects of Sildenafil, Bosentan and combined therapy in patients with congenital cardiac shunts associated pulmonary artery hypertension (CCS-PAH). Design: Prospective observational study (February 2011 - January 2014) with a historical control group (January 2009 - January 2011). Setting: "CC Iliescu" Institute for Emergency Cardiovascular Diseases of Bucharest, a tertiary university-affiliated center. Patients: All cases with CCS-PAH. Interventions: Specific vasodilatory therapy: Sildenafil, Bosentan or combined therapy. Outcome Measures: The primary outcome was the overall survival at 24 months. Results: Out of 108 patients with pulmonary arterial hypertension, there were 79 patients with CCS-PAH, 55 presenting a severe form of the disease. The mean age of the patients was 34.42±21.15 years, with 37 (67,3%) female patients. 23 patients received specific vasodilatory treatment (thirteen Sildenafil, seven Bosentan, three combined treatment), with 32 patients in the control group, without specific vasodilatory therapy. The specific vasodilatory therapy was associated with improved WHO/ NYHA functional class (p=0.025), oxygen saturation at the end of the six-minute walk test (p=0.011), decreased pulmonary artery systolic (p=0.002) and diastolic (p=0.004) pressures, and an increased S' wave in Tissue Doppler Imaging (p=0.008). Conclusions: Despite the complexity of CCS-PAH, with a complex constellation of underlying congenital heart defects, there are short-term benefits of a specific vasodilatory therapy.