Initial dual oral combination therapy for pulmonary arterial hypertension (PAH) in daily practice

Abstract

There is little data on the use of initial dual oral combination therapy (Initial Combo) for PAH in current practice. Methods: Clinical and haemodynamic data from newly diagnosed PAH patients on Initial Combo (endothelin receptor antagonist and PDE-5 inhibitor) were collected at baseline and at first evaluation (3 to 6 months) from the prospective French PAH registry. We compared the overall survival to the expected survival according to the French equation ( Humbert. Eur Respir J 2010 ). Results: From 2007 to 2013, 52 patients (54±18 years; F:M 37:15), were included. Most of them had idiopathic/heritable PAH (n=29) or PAH associated with connective tissue disease (n=12). Forty patients (77%) received a combination of bosentan and sildenafil. After 3-6 months, significant improvements in functional class, exercise capacity and haemodynamics were seen (Table). Mean follow-up was 40±21 months. Nine patients died and 15 required treatment intensification. Actual survival rates were 95%, 86% and 82%, at 1, 2 and 3 years, compared to expected survival rates of 86%, 75% and 67%. Conclusion: Initial dual oral combination therapy in patients with PAH reduces PVR by 40% at 3-6 months, and is associated with an 82% survival rate at 3 years. An initial combination therapy strategy is appealing and needs to be properly compared to sequential combination therapy.