Features of therapy and frequency of adverse events in patients with pulmonary hypertension of different etiology

Objective: to evaluate the characteristics of PAH-specific and symptomatic therapy, treatment escalation dynamics, and the incidence of adverse events over a 3-year follow-up period in patients with pulmonary hypertension (PH) of various etiologies. Materials and methods: the study included patients with PH of different etiologies: idiopathic pulmonary arterial hypertension (IPAH, n=30), PH associated with congenital heart disease (CHD, n=30), and chronic thromboembolic pulmonary hypertension (CTEPH, n=31). We analyzed the duration and structure of PAH-specific therapy, as well as the incidence of adverse events. Statistical analysis was performed to assess intergroup and pairwise differences, which were considered statistically significant at p < 0.05. Results: Results: analysis of PAH-specific therapy at baseline revealed that prior to study enrollment, 77% of patients with CTEPH had not received any PAH-specific therapy, which was significantly more frequent than in the CHD group (p₃=0,004). Dual therapy prior to enrollment was more commonly used in patients with CHD (20%), whereas it was absent in the IPAH and CTEPH groups (p_mg=0,004). Following therapy escalation, differences regarding the absence of PAH-specific treatment were no longer significant; however, treatment patterns differed: dual therapy remained more frequent in the CHD group (37%; p_mg=0,017), sildenafil was more frequently prescribed to patients with CHD (70%; p_mg=0,003), and riociguat was more frequently prescribed to patients with CTEPH (55%; p_mg <0,001). At the 3-year follow-up, patients with IPAH showed a significant increase in the proportion receiving triple therapy (p₂ =0,001; p_mg=0,007), patients with CHD showed a decrease in monotherapy and an increase in dual therapy (p₂ =0,007 and p₂ =0,005, respectively), whereas no significant changes in the structure of PAH-specific therapy were observed in the CTEPH group. Analysis of adverse events revealed the highest 3-year mortality rate in the IPAH group (16%). At 1 year, the composite endpoint (all-cause mortality and >1 hospitalization per year) was more frequently observed in patients with IPAH and CTEPH (p_mg=0,004). Conclusion: in patients with IPAH, treatment escalation was characterized by an increase in triple therapy regimens; in CHD-associated PH, by a shift from monotherapy to dual therapy; and in CTEPH, monotherapy with riociguat predominated. These findings support the need for an etiology-specific approach to PH management. Adverse events were more frequently reported in patients with IPAH and CTEPH.

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