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Comparative Study
. 2024 Nov 27;25(12):1712-1720.
doi: 10.1093/ehjci/jeae177.

Prognostic utility of exercise cardiovascular magnetic resonance in patients with systemic sclerosis-associated pulmonary arterial hypertension

James T Brown  1   2   3 , Ruta Virsinskaite  1   2   3 , Tushar Kotecha  1   2   3 , Jennifer A Steeden  3 , Marianna Fontana  2   4 , Nina Karia  1   2   3 , Benjamin E Schreiber  1 , Voon H Ong  5 , Christopher P Denton  5 , J Gerry Coghlan  1 , Vivek Muthurangu  3 , Daniel S Knight  1   2   3
Affiliations
Comparative Study

Prognostic utility of exercise cardiovascular magnetic resonance in patients with systemic sclerosis-associated pulmonary arterial hypertension

James T Brown et al. Eur Heart J Cardiovasc Imaging. .

Abstract

Aims: Systemic sclerosis complicated by pulmonary arterial hypertension (SSc-PAH) is a rare condition with poor prognosis. The majority of patients are categorized as intermediate risk of mortality. Cardiovascular magnetic resonance (CMR) is well placed to reproducibly assess right heart size and function, but most patients with SSc-PAH have less overtly abnormal right ventricles than other forms of PAH. The aim of this study was to assess if exercise CMR measures of cardiac size and function could better predict outcome in patients with intermediate risk SSc-PAH compared with resting CMR.

Methods and results: Fifty patients with SSc-PAH categorized as intermediate risk underwent CMR-augmented cardiopulmonary exercise testing. Most patients had normal CMR-defined resting measures of right ventricular (RV) size and function. Nine (18%) patients died during a median follow-up period of 2.1 years (range 0.1-4.6). Peak exercise RV indexed end-systolic volume (ESVi) was the only CMR metric to predict prognosis on stepwise Cox regression analysis, with an optimal threshold < 39 mL/m2 to predict favourable outcome. Intermediate-low risk patients with peak RVESVi < 39 mL/m2 had significantly better survival than all other combinations of intermediate-low/-high risk status and peak RVESVi< or ≥39 mL/m2. In our cohort, ventilatory efficiency and resting oxygen consumption (VO2) were predictive of mortality, but not peak VO2, peak cardiac output, or peak tissue oxygen extraction.

Conclusion: Exercise CMR assessment of RV size and function may help identify SSc-PAH patients with poorer prognosis amongst intermediate risk cohorts, even when resting CMR appears reassuring, and could offer added value to clinical PH risk stratification.

Keywords: cardiopulmonary exercise testing; cardiovascular magnetic resonance; exercise; prognosis; pulmonary arterial hypertension; systemic sclerosis.

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Conflict of interest statement

Conflict of interest: There are no conflicts of interest in relation to the work in this manuscript. Non-conflicting relationships with industry are detailed as follows: R.V. reports support for attending meetings and travel from Janssen. T.K. reports speaker bureau fees and support for attending meetings and travel from Janssen and Inari. M.F. reports consulting board fees from Alnylam, Alexion, AstraZeneca, Bridgbio/Eidos, Prothena, Attralus, Intellia, Ionis, Cardior, Lexeo, and Pfizer, and research grants from Alnylam, AstraZeneca, Bridgbio, and Pfizer. N.K. reports advisory board fees and support for attending meetings and travel from Janssen. V.H.O. reports speaker fees and support for attending meetings and travel from Boehringer Ingelheim. C.P.D. reports consultancy fees from Janssen, GlaxoSmithKline, Bayer, Sanofi, Boehringer Ingelheim, Roche, CSL Behring, Corbus, Acceleron, Horizon, Arxx, Lilly, Novartis, and Certa, speaker fees from Janssen, GlaxoSmithKline, and Boehringer Ingelheim, and grants from CSL Behring, GlaxoSmithKline, and Abbvie. J.G.C. reports consulting and speaker bureau fees from Bayer, GlaxoSmithKline, Inari, Janssen, and Merck Sharp & Dohme, support for attending meetings and travel from Janssen, and research funding from Janssen. D.S.K. reports speaker and advisory board fees, research funding, and support for attending meetings and travel from Janssen.

Figures

Graphical Abstract
Graphical Abstract
Figure 1
Figure 1
Forest plot of resting CMR and CPET variables to predict all-cause mortality on univariable Cox regression analysis. Asterisk denotes statistically significant metrics (P < 0.05).
Figure 2
Figure 2
Forest plot of peak exercise CMR and CPET variables to predict all-cause mortality on univariable Cox regression analysis. Asterisk denotes statistically significant metrics (P < 0.05).
Figure 3
Figure 3
Kaplan–Meier plots showing: (A) survival curves for reduced and normal right ventricular contractile reserve groups, as defined by a peak RVESVi threshold of <39 mL/m2; (B) survival curves for four groups defined by all combinations of reduced and normal RV contractile reserve and intermediate-low/-high risk.
See this image and copyright information in PMC

References

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