Open Access

ARTICLE

Impaired Magnetic Resonance Myocardial Strain in Unoperated Ebstein’s Anomaly Is Associated with Reduced Exercise Capacity

Ahmed M. Dardeer^1,2,3,#, Victoria M. Stoll^1,2,#, Boyang Liu^1,2, William E. Moody^1,2, Colin D. Chue¹, Paul Clift^1,2, Roman Wesolowski⁴, Lucy E. Hudsmith¹, Richard P. Steeds^1,2,*

1 Department of Cardiology, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust, Birmingham, B15 2GW, UK
2 Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, B15 2TT, UK
3 Department of Cardiology, Faculty of Medicine, Minia University, Minia, 61519, Egypt
4 RRPPS, Department of Imaging and Medical Physics, University Hospitals Birmingham NHS Foundation Trust, Birmingham, B15 2GW, UK

* Corresponding Author: Richard P. Steeds. Email:
# Ahmed M. Dardeer and Victoria M. Stoll are joint first authors, both contributed equally to the work

Congenital Heart Disease 2025, 20(1), 27-39. https://doi.org/10.32604/chd.2025.059729

Received 15 October 2024; Accepted 13 February 2025; Issue published 18 March 2025

Abstract

Background: Patients with unrepaired Ebstein’s anomaly experience exercise intolerance, heart failure and premature mortality. Volumetric assessment of right ventricular function is difficult due to the complex anatomy of the right ventricle and tricuspid valve. Myocardial deformation indices are an early marker in other cardiac pathologies of ventricular dysfunction. Objectives: 1. Assess myocardial deformation in unrepaired Ebstein’s compared to healthy controls. 2. Investigate the relationships between myocardial deformation and exercise capacity. Methods: Myocardial deformation parameters (strain) were calculated using feature tracking from standard cardiac magnetic resonance cine images. Cardiopulmonary exercise results were included where available. Results: 36 patients with unrepaired Ebstein’s and 36 matched controls were included. Right ventricular, right atrial, and left ventricular global longitudinal, as well as left ventricular circumferential strain were impaired in Ebstein’s patients compared to controls (p < 0.05). In Ebstein’s patients right atrial peak strain correlated with their percentage predicted VO₂ max (r = −0.448, p = 0.022) and VE/VCO₂ slope (r = 0.435, p = 0.026). There were no correlations between right ventricular ejection fraction and exercise parameters. When Ebstein’s patients were divided by severity into mild or severe according to the median total right/left index, those with severe demonstrated significantly impaired right ventricular global longitudinal strain compared to those in the mild category (−17.5 ± 5.4% vs. −21.4 ± 4.4%, p = 0.0017). Conclusions: Myocardial deformation parameters for both the right and left ventricle are impaired in patients with unrepaired Ebstein’s compared to healthy controls. Right atrial peak strain is related to impaired exercise capacity and warrants further investigation as an early prognostic marker in this patient cohort.

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