The clinical profile of pulmonary arterial hypertension in newly diagnosed patients with connective tissue disease from the point of view of the cardiologist

Abstract

Aim: to evaluate demographic and disease characteristics in pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) patients.Materials and methods: the study enrolls 68 patients with PAH-CTD. These patients were diagnosed in department of pulmonary hypertension and heart disease of the E.I. Chazov National Medical Research Centre of cardiology. Clinical, functional and hemodynamic characteristics of PAH patients were examined. The diagnosis was confirmed according to Eurasian (2019) and Russian (2020) guidelines for the diagnosis and treatment of pulmonary hypertension.Results: Of 68 patients with CTD 35 (51,5%) had systemic sclerosis, 11 (16,2%) - mixed CTD, 22 (32,3%) - other CTD. The median time from PAH onset to first visit to health care worker was 4 months, the median time from first visit to PAH diagnosis was 7,5 months. The median age was 59,5 years, 94,1% patients were women. The median 6-minute walking distance (6MWD) was 345 (259,25-400,00) m, which was corresponding to WHO functional class III, the median Borg dyspnea index was 5 (3,0-6,0). 12 (17,7%) patients did not undergo 6MWD due to severity of their condition. According to right heart catheterization data the median mean pulmonary arterial pressure was 51,0 (35,5-62,0) mmHg, the median cardiac index was 2,1 (1,7-2,5) liter/min/m2, the median pulmonary vascular resistance was 11,48 (5,99-15,71) Wood units. According to several risk stratification calculators, the vast majority of patients were at high risk of 1-year mortality: due to risk status, PAH-specific therapy was initiated to 88,3% patients: 42,7% received double and 8,8% - triple PAH-specific therapy.Conclusions: In Russian clinical practice, PAH-CTD is characterized by late- onset diagnosis. Newly diagnosed patients have significant functional and hemodynamic impairment, high risk of 1-year mortality, which results in combined PAH-specific therapy initiation.