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. 2025 Feb;38(2):115-126.
doi: 10.1016/j.echo.2024.09.010. Epub 2024 Oct 1.

Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease

Abhishek Gami  1 , Vivek P Jani  1 , Hoda Mombeini  2 , Ryan Osgueritchian  2 , Ilton M Cubero Salazar  2 , Matthew Kauffman  3 , Catherine E Simpson  3 , Rachel L Damico  3 , Todd M Kolb  3 , Ami A Shah  4 , Stephen C Mathai  3 , Ryan J Tedford  5 , Steven Hsu  2 , Paul M Hassoun  3 , Monica Mukherjee  6
Affiliations

Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease

Abhishek Gami et al. J Am Soc Echocardiogr. 2025 Feb.

Abstract

Background: Ineffective right ventricular (RV) adaptation to increasing pulmonary arterial (PA) afterload in pulmonary vascular disease (PVD) significantly contributes to morbidity and mortality. Pulmonary vascular disease in systemic sclerosis (SSc) arises through various mechanisms, yet detecting abnormal contractile response remains challenging. Here we examine whether echocardiographic RV-PA coupling metrics correlate with invasive pressure-volume (PV) loops, enhancing the prediction of adverse clinical outcomes in SSc-PVD patients.

Methods: Prospectively enrolled patients with SSc-PVD with paired echocardiogram and PV loops were included. Linear regression and receiver-operating curve analysis were used to assess the relationship between tricuspid annular plane systolic excursion/PA systolic pressure (PASP), fractional area change/PASP, tissue Doppler velocityS'/PASP, and RV free wall strain (RVFWS)/PASP and coupling thresholds defined by end-systolic to end-arterial elastance (Ees/Ea), obtained by the multibeat method. The contribution of right atrial strain (RAS) to RV-PA coupling parameters was also investigated. Kaplan-Meier analysis was used to identify the relationship between coupling ratios and composite outcomes including clinical worsening, lung transplant, and death.

Results: Forty-two patients with SSc were studied, 91% female, with a mean age of 59 ± 12 years and varying degrees of PVD: mean pulmonary artery pressure 29.5 ± 12.8 mm Hg, PVR 4.7 ± 4.2 WU, and PCWP 10.3 ± 4.1 mm Hg. Echocardiographic coupling metrics including tricuspid annular plane systolic excursion/PASP, fractional area change/PASP, tissue Doppler velocity S'/PASP, RVFWSglobal and RVFWSbasal/PASP, and RASreservoir/PASP were linearly associated with Ees/Ea. At cut points obtained through receiver-operating curve analysis, all ratios were predictive of RV-PA uncoupling, defined by Ees/Ea, and composite outcomes. Additionally, RASreservoir/RVFWS correlated with Ees/Ea even after adjustment for PASP, suggesting that diminished RAS further impacts RV performance and coupling.

Conclusion: Echocardiographic RV-PA coupling ratios strongly correlate with invasive Ees/Ea and predict adverse clinical outcomes in SSc patients across the spectrum of PVD. Further, we demonstrate how RAS impacts RV performance. These findings may refine risk stratification and prognostication in this at-risk cohort.

Keywords: Mortality; Pulmonary hypertension; Right atrium; Right ventricle; Systemic sclerosis.

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Conflict of interest statement

Disclosures Dr. Tedford is the cochair of the PH due to left heart disease task force for 7th World Symposium on Pulmonary Hypertension and deputy editor for the Journal of Heart and Lung Transplantation. He reports general disclosures to include consulting relationships with and receiving honoraria from Abbott, Acorai, Aria CV Inc., Acceleron/Merck, Alleviant, Boston Scientific, Cytokinetics, Edwards LifeSciences, Endotronix, Gradient, Medtronic, Morphic Therapeutics, Restore Medical, and United Therapeutics. Dr. Tedford serves on the steering committee for Abbott, Edwards, Endotronix, and Merck as well as a research advisory board for Abiomed. He also does hemodynamic core lab work for Merck. The remaining authors have nothing to disclose.

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