Risk assessment and survival of patients with pulmonary hypertension: Multicenter experience in Turkey.

Abstract

Objective:Risk stratification continues to evolve in pulmonary arterial hypertension (PAH). Our aim was to further confirm the risk assessment strategy in our cohort and to determine the most reliable model.Methods:We enrolled incident patients with idiopathic PAH (IPAH), heritable, drug-induced, congenital heart disease (CHD), connective tissue diseases (CTD) subsets, and chronic thromboembolic pulmonary hypertension (CTEPH) from January 2008 to February 2018. Data from the baseline and subsequent follow-ups within 1 year of diagnosis were included. An abbreviated risk assessment strategy was applied using the following variables: functional class (FC), 6-minute walk distance (6 MWD), N-terminal pro–brain natriuretic peptide (NT-proBNP) or BNP, right atrial (RA) area, pericardial effusion, the mean RA pressure, cardiac index, and mixed venous oxygen saturation. Three different methods were applied to categorize patients.Results:A total of 189 subjects (46±17 years, 23% male) were included. Sixty-one patients had died. The survival differed significantly between the risk groups both at diagnosis and during the follow-up. Patients with a low-risk profile had a better survival rate. An abbreviated risk assessment tool predicted mortality at early follow-up in the entire group and CHD, CTD subsets, and CTEPH, separately. An overall mortality among risk categories was significantly different according to each categorization method. The most reliable model comprised FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up.Conclusion:The abbreviated risk assessment tool may be valid for the PAH subsets and CTEPH. Echocardiographic variables do matter. A model comprising FC, 6 MWD, NT pro-BNP/BNP, and the RA area at the follow-up could be useful for better prognostication.