Key result
Lack of clinical and RV-PA coupling improvement in pulmonary hypertension is linked to ~3.1-fold higher mortality.
Why the study?
Do time-dependent improvements in clinical, risk stratification, and echocardiographic RV-PA coupling measures reduce all-cause mortality across pulmonary hypertension groups?
Cohort (n=1,213)
No
Do time-dependent improvements in clinical, risk stratification, and echocardiographic RV-PA coupling measures reduce all-cause mortality across pulmonary hypertension groups?
Serial assessments in pulmonary hypertension patients demonstrate that treatment-related improvements in functional class, 6MWD, multiparametric risk scores, and echocardiographic RV-PA coupling at 6 and 12 months are significantly associated with improved survival.
Aims In this study, we evaluated the prognostic impact of the time-dependent changes in the clinical and echocardiographic measures for survival across the pulmonary hypertension (PH) groups and subgroups. Methods This single-center retrospective study included 1,213 patients with PH. Patients underwent periodic follow-up (FU) at 3 to 6-month intervals, including clinical evaluations, echocardiography, and risk stratification using ESC/ERS, REVEAL 2.0, REVEAL lite 2.0, and FPHN models. The primary endpoint was all-cause mortality. Results During a median FU of 616 days (IQR: 135–1900), 5-year survival rates for Groups 1, 2, 3, and 4 were 56.9%, 40.6%, 27.4%, and 51.9%, respectively (p<0.001). Clinical deterioration and survival curve separation became most evident within the first 6–12 months. Compared to PAH associated with congenital heart disease (APAH-CHD), mortality risk was significantly higher in PAH associated with connective tissue disease (APAH-CTD; HR=3.137, 95% CI: 2.118–4.646, p<0.001) and idiopathic PAH (IPAH; HR=1.608, p<0.001). Significant longitudinal reductions were observed in pulmonary artery systolic and mean pressures (PASP/PAMP) across the cohort (p<0.001 and p=0.009). Group 4 exhibited lower baseline PASP/PAMP and more pronounced reductions with treatment compared to Group 1. Although APAH-CHD maintained higher PASP throughout FU (p<0.001), the APAH-CTD group showed the most dramatic PASP reduction (p<0.001). Right ventricular (RV) function and RV-PA coupling (TAPSE, S’, and TAPSE/PASP ratio) significantly improved in Group 1 (p<0.001). Improvements in functional class, 6MWD, risk status via MRS, and RV-PA coupling measures at 6 and 12 months were all significantly associated with reduced mortality (p=0.011 to p<0.001). Conclusion In our PH cohort, serial assessments revealed that improvements in FC, 6MWD, MPRs and right ventricle- PA coupling with targeted therapies can be translated to improved survival.
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Kaymaz et al. (2026) conducted a cohort in Pulmonary hypertension (n=1,213). Pulmonary hypertension group and time-dependent changes in clinical, risk, and RV-PA coupling measures vs. Across PH groups (Groups 1-4) and PAH subgroups (e.g., APAH-CTD vs. APAH-CHD) was evaluated on All-cause mortality (HR 3.137, 95% CI 2.118-4.646, p=<0.001). Improvements in functional class, 6MWD, risk status, and RV-PA coupling at 6-12 months were associated with reduced mortality in pulmonary hypertension (p=0.011 to p<0.001).
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