10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicenter PAH registry.

Abstract

To report the 10-year survival rate and prognostic factors of pulmonary arterial hypertension associated with connective tissue disease (CTD-PAH) patients, to compare treatment and survival between patients enrolled before and after 2015, and to validate the discrimination of the recommended 4-strata model in predicting 10-year survival at follow-up in Chinese CTD-PAH patients. This study was derived from a Chinese national multicentre prospective registry study from 2009 to 2019. Medical records were collected at baseline and follow-up, including PAH-targeted therapy and binary therapy (both CTD and PAH-targeted therapy). A total of 266 CTD-PAH patients were enrolled and the 10-year survival rate was 59.9% (median follow-up time: 4.85 years). Underlying CTD (SSc), baseline 6-min walking distance and SaO2 were independent risk factors for 10-year survival. The proportion of patients receiving PAH-targeted combination therapy increased from 10.1% (2009-2014)-26.5% (2015-2019) and that of binary therapy increased from 14.8% to 35%. The 1-year survival rate increased from 89.8% (2009-2014)-93.9%, and the 3-year survival rate increased from 80.1% (2009-2014)-86.5% (both p > 0.05). The 4-strata strategy performed well in predicting 10-year survival at follow-up (C-index = 0.742). The 10-year survival rate of CTD-PAH patients was reported for the first time. The 10-year prognosis was poor, but there was a tendency for more standardized treatment and better survival in patients enrolled after 2015. The recommended 4-strata model at follow-up can effectively predict 10-year survival in CTD-PAH patients.