O57-5 - Clinical Features of the Systemic Sclerosis Associated Pulmonary Arterial Hypertension with Left Heart Disease

Abstract

Left heart disease (LHD) is common in systemic sclerosis (SSc). Although LHD is supposed to associate with increased mortality, there was little data as to the LHD in the patients with pulmonary arterial hypertension (PAH) associated with SSc. 25 patients with PAH associated with SSc were retrospectively evaluated. 15 out of 25 patients had LHD, which was defined as TDI-e' (sep) <6 cm/s, left ventricular hypertrophy, valvular heart disease or ischemic heart disease. No patients showed LV systolic dysfunction. LHD group showed advanced age. In the hemodynamic parameter before PAH targeted therapy, LHD group showed lower cardiac index (CI) and higher pulmonary vascular resistance (PVR) compared with non-LHD group. All of the patients were treated with PAH targeted therapy, and the number of PAH targeted therapy was not different in two groups. Although mPAP and PVR were decreased after PAH targeted therapy in both groups, LHD group showed higher mPAP and PVR than non-LHD group. However, prognostic parameters such as survival rate, hospitalization, and frequency of pulmonary edema were not different between two groups. Our data suggested that LHD showed no impact to the prognosis in the PAH patients with SSc, in spite of the high frequency of LHD in PAH patients associated with SSc, and the poor improvement in hemodynamic parameters with the PAH targeted therapy.