Abstract

Pulmonary arterial hypertension (PAH) associated with systemic connective tissue diseases (SCTD) is a poor prognostic manifestation of the latter that result in death if untreated. The invasive determination of hemodynamic parameters is prominent in diagnosing the disease and determining its treatment policy and prognosis. Objective : to analyze the results of catheterization in PAH-SCTD patients admitted to the V.A. Nasonova Research Institute of Rheumatology. Subjects and methods . The investigation included 59 patients admitted to the V.A. Nasonova Research Institute of Rheumatology from September 2009 to September 2014. PAH was diagnosed in accordance with the conventional guidelines. All the patients underwent right heart and pulmonary artery (PA) catheterization at the diagnosis and over time during treatment. Results and discussion. All the patients included in the trial met the pre-capillary pulmonary hypertension (PH) criteria: mean pulmonary artery pressure (MPAP) ≥25 mm Hg; and PA wedge pressure (PAWP) 15 mm Hg and pulmonary vascular resistance (PVR) >3 Wood units could diagnose PAH in all our patients. There was a statistically highly significant association between pathological hemodynamic changes and functional class (FC). FC was found to be most closely correlated with right atrial pressure (RAP), cardiac output (CO), PVR, and cardiac index (CI). Among the most common manifestations of heart failure, only the presence of peripheral edemas was associated with worse hemodynamic parameters in PAH. It should be noted that out of two biomarkers (N-terminal pro-brain natriuretic peptide and uric acid), the former is largely related to the magnitude of changes in hemodynamic factors. The critical values of hemodynamic parameters were due to extreme edema – anasarca (RAP >17 mm Hg, PVR >20 Wood units, CI <14 ml/m2). Analysis of clinical and instrumental parameters in relation to FC revealed a linear relationship of RAP, CO, and PVR between the level of these parameters and FC; moreover, the highest correlation coefficients were observed for the hemodynamic parameters characterizing right ventricular systolic function. It is remarkable that no MPAP changes were found; only patients with FC IV showed its slight increase (from 51±8 to 55±8 mm Hg; р = 0.087). PAWP remained unchanged regardless of FC. Conclusion. Thus, the hemodynamic parameters determined in patients with PAH-SCTD during right heart and LA catheterization are closely related to the manifestations of respiratory and heart failure, the biomarkers, and FC of PAH.

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