Evidence map›Paper›PMID 42252465›Full record

ArticleEcho research and practice2026

Echocardiographic characterization of combined postcapillary and precapillary pulmonary hypertension in systemic sclerosis with preserved ejection fraction.

Ahmad Daoud, Garrett Goldin, Lea Goren, Arindam Bagga, Hoda Mombeini, Ryan Osgueritchian, Vivek P Jani, Steven Hsu, Fredrick M Wigley, Matthew R Lammi and 4 more

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Article in Echo research and practice, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

14 authors.

Ahmad DaoudDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA.
Garrett GoldinDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA.
Lea GorenJohns Hopkins University School of Medicine, Baltimore, MD, USA.
Arindam BaggaJohns Hopkins University School of Medicine, Baltimore, MD, USA.
Hoda MombeiniDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA.
Ryan OsgueritchianDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA.
Vivek P JaniJohns Hopkins University School of Medicine, Baltimore, MD, USA.
Steven HsuDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA.
Fredrick M WigleyJohns Hopkins University Division of Rheumatology, Baltimore, MD, USA.
Matthew R LammiDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
Paul M HassounDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
Ami A ShahJohns Hopkins University Division of Rheumatology, Baltimore, MD, USA.
Stephen C MathaiDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
Monica MukherjeeDivision of Cardiology, Johns Hopkins University, 600 N. Wolfe Street Carnegie 536 |, Baltimore, MD, 21287, USA. mmukher2@jh.edu.

Funding

Early Detection of Right Ventricular Dysfunction and Emerging Pulmonary Hypertension in Systemic SclerosisR01HL162851 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI Monica Mukherjee · 2023 to 2026
$2.5M
Myocyte Mechanics of Right Ventricular Contractile Failure in Pulmonary Hypertension due to Heart Failure with Reduced Ejection FractionR01HL172830 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI Steven Hsu · 2024 to 2026
$2.0M
Harnessing multivariate patient- and population-level disease trajectories to predict major clinical events in sclerodermaK24AR080217 · NIAMS · JOHNS HOPKINS UNIVERSITY · PI Ami Aalok Shah · 2022 to 2026
$847k
Congressionally Directed Medical Research Programs PR191839Congressionally Directed Medical Research Programs PR231648NHLBI NIH HHS R01HL162851NHLBI NIH HHS R01HL172830NIAMS NIH HHS K24080217NIAMS NIH HHS K24 AR080217
6 · The paper itself

Abstract

backgroundSystemic sclerosis (SSc) is a multisystem autoimmune disease frequently complicated by pre- and post-capillary pulmonary hypertension (PH). Within SSc, progressive diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are key contributors, often presenting as isolated postcapillary PH (Ipc-PH) or combined pre- and postcapillary PH (Cpc-PH). The ability to differentiate these hemodynamic phenotypes is critical for risk stratification, yet echocardiographic markers specific to each subtype in SSc-HFpEF are poorly defined.

methodsWe investigated 147 adults with SSc-HFpEF with echocardiograms and right heart catheterization (RHC) assessments performed within one year. Patients were classified as Ipc-PH (n = 46) or Cpc-PH (n = 101) based on guideline-defined hemodynamic criteria. Echocardiographic parameters, including conventional measures, strain indices, and coupling metrics were analyzed. A random forest (RF) classifier was used to identify top echocardiographic predictors of Cpc-PH, and further assessed using multivariable logistic regression. Separate RF models and Cox regression analyses were used to determine echocardiographic predictors of mortality within each group. Survival was assessed using Kaplan-Meier analysis.

resultsPatients with Cpc-PH exhibited significantly greater right heart remodeling, higher pulmonary pressures, and impaired right ventricle (RV)-pulmonary artery (PA) coupling. The top echocardiographic predictors of Cpc-PH included reduced RV free wall strain (RVFWS), decreased RVFWS/PA systolic pressure (PASP) and fractional area change (FAC)/PASP ratios, elevated PASP, lower septal e′ velocity, and higher systolic LV eccentricity index (LV EI). In adjusted Cox models, elevated LVEI (HR 1.39), increased RV internal diastolic diameter (HR 2.15), and reduced left and right atrial strain (HR 0.91 and 0.94, respectively) were independently associated with mortality in Cpc-PH. In Ipc-PH, mortality was linked to reduced FAC/PASP, lower left ventricular global longitudinal strain (LVGLS), increased LV mass index, elevated PASP, and lower RVFWS.

conclusionIn the present study, we demonstrate key echocardiographic differences between Ipc-PH and Cpc-PH within the SSc-HFpEF population, emphasizing the central role of right heart remodeling, RV-PA coupling, atrial and septal mechanics in phenotypic differentiation and prognostication. Strain-based parameters and RV-PA coupling indices offer incremental value for risk stratification and may guide more tailored therapeutic strategies in this heterogeneous population.

Indexed as

EchocardiographyHeart failure with preserved ejection fractionPulmonary hypertensionSystemic sclerosis

Identifiers

PMID42252465
PMCPMC13244854

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.