Survival and prognostic factors in patients with connective tissue disease‐associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry

Abstract

In Korean patients with CTD-PH, the 3-year survival rate was 87%. Low diffusion capacity of carbon monoxide (DLCO), pleural effusion and diabetes mellitus were independent poor prognostic factors. Anti-UI-RNP antibody was protective. Prompt PAH-specific vasodilator therapy may improve the survival of patients with severe CTD-PH.