Mortality of idiopathic / hereditary and connective tissue disease-associated pulmonary arterial hypertension : the data from first single-center prospective registry in Indonesia

Most pulmonary arterial hypertension (PAH) in Indonesia is associated with uncorrected congenital heart disease (CHD). Other type of PAH has not been reported from Indonesia, despite current availability of PAH-specific medication. The aim of this study is to describe the characteristics of nonCHD-associated PAH and its mortality. We had established the COHARD-PH registry which is a prospective singlecenter study enrolling adult patients with CHD-associated PAH. We also enrolled and followed subjects with nonCHD-PAH into separate cohort. Layers of diagnostic procedures were performed from echocardiography, HRCT thorax, MSCT pulmonary angiography and right heart catheterization (RHC). The baseline data were collected during the index of diagnosis of PH probability by echocardiography. The RHC was performed to diagnose PAH. Subjects were followed up during their visit to our PH clinic. Sixty-three patients were enrolled, 57 (90.5%) were idiopathic/hereditary PAH (I/H-PAH), five (7.9%) connective tissue disease-associated PAH (CTD-PAH) and one portopulmonary PAH (po-PAH). Most subjects were young females (I/H-PAH 82.5%, 39.0 14.0 years and CTDPAH 80.0%, 41.2 6.3 years). Most subjects had WHO functional class II. The RHC was performed in 26 I/H-PAH subjects, with mean mPAP 62.3 18.7mmHg and PVRi 29.2 14.0 WU. m. Most treatment was sildenafil monotherapy (71%) and sildenafilþberaprost combination (12.7%). During follow-up, 12 (19.1%) subjects died. Those who died were younger (32.6 10.8 years vs. 41.1 13.7 years, p< 0.05). Subjects with CTD-PAH had the highest mortality rate (two subjects (40%) and I/H-PAH had 15.8% mortality rate (nine subjects)). The only poPAH subject died during follow-up. Among I/H-PAH and CTD-PAH, higher right atrial diameter by echocardiography significantly associated with mortality. In I/H-PAH, mortality had tendency toward higher mPAP and PVRi. In conclusion, among non CHD-associated PAH, CTD-PAH had worse outcome as compared with I/H-PAH. Younger age and higher right atrial diameter by echocardiography were significantly associated with mortality in I/H-PAH and CTD-PAH.