Complex assessment of clinical, functional and hemodynamic status in patients with incident pulmonary arterial hypertension in real clinical practice

Objective. The complex assessment of clinical, functional and hemodynamic status change according to echocardiography and right heart catheterization (RHC) within 12 months after pulmonary arterial hypertension (PAH) verification in real clinical practice. Design and methods. Incident adult patients with PAH were included in a longitudinal prospective observational study with a retrospective component after PAH verification based on RHC. The data were received by patients interviewing and medical documentation analysis at enrollment and every 3 months during one-year observational period. The retrospective design was used to collect information before enrollment. Results. Thirty three patients were enrolled. 64 % of patients received monotherapy as initial PAH-specific therapy, 24 % — a double combination, while the rest received calcium channel blockers. Follow-up echocardiography was performed in 75,8 % patients. There was a significant increase in median tricuspid annular plane systolic excursion (TAPSE, p = 0,001), median left ventricular end-diastolic diameter (p = 0,004) and a decrease in the anteroposterior diameter of the left atrium (p = 0,016) during the follow-up period. There were no significant changes in the size of the right heart chambers, pulmonary artery systolic pressure, or pulmonary artery size. Follow-up RHC was performed in 16 (48,5 %) patients. There was a significant improvement in cardiac output (p = 0,006) and cardiac index (p = 0,012), venous blood oxygen saturation (p = 0,045) and significant decrease of stroke volume (p = 0,021) during the follow-up period. The initial 6-minute walk test was performed in 75,8 % patients, the median distance was 340 m. During follow-up data were available in 14 patients, the median distance was 398 m. Median time from PAH onset to PAH verification was 13,5 months with a direct correlation between the time from the PAH onset to PAH verification and WHO functional class at the time of diagnosis (ρ = 0,394, p < 0,05). Within 12 months after PAH verification most patients subjectively noted the absence of positive dynamics in symptoms (88 %). Conclusions. We suppose that optimizing the treatment regimen for patients with PAH, improving access to PAH-specific therapy can improve the clinical and hemodynamics status and prognosis of patients with PAH.

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