Evidence map›Paper›PMID 40744380›Full record

ArticleJournal of the American Society of Echocardiography : official publication of the American Society of Echocardiography2025

Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.

Ryan Osgueritchian, Vivek P Jani, Hoda Mombeini, Abhishek Gami, Garrett Goldin, Steven Hsu, Laura K Hummers, Fredrick M Wigley, Matthew R Lammi, Bharath Ambale-Venkatesh and 4 more

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Article in Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Review
  4. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

14 authors.

Ryan OsgueritchianDivision of Cardiology, Johns Hopkins University, Baltimore, Maryland.
Vivek P JaniJohns Hopkins University School of Medicine, Baltimore, Maryland.
Hoda MombeiniDivision of Cardiology, Johns Hopkins University, Baltimore, Maryland.
Abhishek GamiJohns Hopkins University School of Medicine, Baltimore, Maryland.
Garrett GoldinDivision of Cardiology, Johns Hopkins University, Baltimore, Maryland.
Steven HsuDivision of Cardiology, Johns Hopkins University, Baltimore, Maryland.
Laura K HummersDivision of Rheumatology, Johns Hopkins University, Baltimore, Maryland.
Fredrick M WigleyDivision of Rheumatology, Johns Hopkins University, Baltimore, Maryland.
Matthew R LammiDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.
Bharath Ambale-VenkateshDepartment of Radiology, Johns Hopkins University, Baltimore, Maryland.
Paul M HassounDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.
Ami A ShahDivision of Rheumatology, Johns Hopkins University, Baltimore, Maryland.
Stephen C MathaiDivision of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.
Monica MukherjeeDivision of Cardiology, Johns Hopkins University, Baltimore, Maryland. Electronic address: mmukher2@jhu.edu.

Funding

Sample Processing and Immunoassay Research CoreP30AR070254 · NIAMS · JOHNS HOPKINS UNIVERSITY · PI Antony Rosen · 2016 to 2026
$8.9M
Mechanisms of Right Ventricular Dysfunction in Scleroderma-associated PAHR01HL114910 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI HASSOUN, PAUL M. · 2012 to 2023
$6.2M
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
NHLBI NIH HHS R01 HL114910NHLBI NIH HHS R01 HL162851NHLBI NIH HHS R01 HL172830NIAMS NIH HHS P30 AR070254
6 · The paper itself

Abstract

backgroundPulmonary arterial hypertension (PAH) confers high morbidity and mortality, particularly in patients with systemic sclerosis (SSc), in which right ventricular (RV) adaptation to pulmonary artery (PA) afterload is a key determinant of outcome. Although several clinical risk scores aid in prognostication, their performance in SSc-associated PAH (SSc-PAH) is unclear. The aims of this study were to evaluate the association of established PAH risk scores with mortality in SSc-PAH and to assess whether echocardiographic RV-PA coupling metrics enhance risk discrimination.

methodsOne hundred seventy-four patients with SSc-PAH from the Johns Hopkins Scleroderma Registry who underwent echocardiography within 6 months of invasive confirmation of PAH status were retrospectively analyzed. Patients were followed up for a median of 60.5 months (interquartile range, 34-104 months). Risk scores assessed included COMPERA 2.0 (four strata), REVEAL 2.0, REVEAL Lite 2, and French noninvasive criteria. Echocardiographic coupling metrics included six-segment RV global longitudinal strain, three-segment RV free wall strain (RVFWS), and segmental RVFWS normalized to PA systolic pressure (PASP). Cox regression and Harrell's C index were used to assess 1-year mortality associations. Enhanced models incorporating coupling parameters into each risk score were compared with the original models using likelihood ratio tests.

resultsThe study cohort (mean age, 63.6 ± 12.7 years) consisted predominantly of white (76.4%) women (85.6) with limited SSc (86%) and moderate to severe SSc-PAH. Risk score C indices ranged from 0.664 to 0.751. Adding RV global longitudinal strain/PASP, RVFWS/PASP, and midventricular RVFWS/PASP significantly improved mortality prediction (P < .05), yielding the greatest increase in C index across all scores.

conclusionsIn SSc-PAH, established risk scores demonstrate modest discrimination for 1-year mortality. Incorporating echocardiographic parameters of RV-PA coupling significantly improves risk assessment, offering a noninvasive approach to refine prognostication in this high-risk population.

Indexed as

EchocardiographyHeart VentriclesHypertension, PulmonaryPulmonary Arterial HypertensionScleroderma, SystemicFemaleFollow-Up StudiesHumansMaleMiddle AgedPrognosisRegistriesRetrospective StudiesRisk AssessmentRisk FactorsSurvival RateEchocardiographyMortalityPulmonary arterial hypertensionRight ventricleScleroderma

Identifiers

PMID40744380
PMCPMC13586975

What OpenQuestion holds

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Registered trials

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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.