Prognostic impact of the time-dependent improvements in outcome measures and right ventricle to pulmonary arterial coupling across pulmonary hypertension groups: a single-center cohort
C Kaymaz, H C Tokgoz, B Kultursay, S Tanyeri, C Bulus, C E Cagliyan, M Kibar, C Erdem, F B Erdogan, O D Ertan, A Kaya, B Keskin, A Hakgor, I H Tanboga, N OzdemirAbstract
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.Figure1For image description, please refer to the figure legend and surrounding text.Figure2For image description, please refer to the figure legend and surrounding text.