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High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young

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Abstract

An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). Myocardial fibrosis measured by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE) also affects prognosis. We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis or perfusion deficit on CMR in HCM. In total 42 individuals (7–31 years); 26 HCM patients, seven genotype-positive, phenotype-negative individuals at risk of HCM (first-degree relatives) and nine healthy volunteers, underwent CMR to identify, and grade extent of, myocardial fibrosis and perfusion defect. 12-lead ECG was used for calculating the ECG risk-score (grading 0–14p). High-risk ECG (risk-score > 5p) occurred only in the HCM group (9/26), and the proportion was significantly higher vs mutation carriers combined with healthy volunteers (0/16, p = 0.008). Extent of LGE correlated to the ECG-score (R2 = 0.47, p = 0.001) in sarcomeric mutations. In low-risk ECG-score patients (0–2p), median percent of myocardium showing LGE (LGE%LVM) were: 0% [interquartile range, IQR, 0–0%], in intermediate-risk (3–5p): 5.4% [IQR 0–13.5%] and in high-risk (6–14p): 10.9% [IQR 4.2–12.3%]. ECG-score > 2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR and 77% vs. 75% to detect perfusion defects in sarcomeric mutations carriers. In patients with myocardial fibrosis as identified by LGE, median ECG risk-score was 8p [range 3–10p]. In conclusions, ECG risk-score > 2 p could be used as a cut-off for screening of myocardial fibrosis. Thus ECG risk-score is an inexpensive complementary tool in risk stratification of HCM in the young.

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Data and material are available after contacting the corresponding author.

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Acknowledgements

We are deeply grateful to the support from the study participants during the study. The authors would like to thank the technicians Ann-Helen Arvidsson and Christel Carlander for their help with CMR data acquisition. Special thanks to research nurse Annika Maxedius for excellent support in the study.

Funding

This study has been possible by support and grants from Region Ostergotland (ALF and the Office for Trainee and Fellowship programs), the Strategic Research Area in Forensic Science, Schelins Foundation and FORSS (Medical Research Council of Southeast Sweden).

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Authors and Affiliations

  1. Crown Princess Victoria Children’s Hospital, Department of Biomedical and Clinical Sciences, Department of Pediatrics, Linköping University, 581 85, Linköping, Sweden

    Anna Wålinder Österberg & Eva Fernlund

  2. Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden

    Ingegerd Östman-Smith

  3. Clinical Physiology, Department of Clinical Sciences, Lund University, Lund, Sweden

    Robert Jablonowski & Marcus Carlsson

  4. Department of Forensic Genetics and Forensic Toxicology, National Board of Forensic Medicine, Linköping, Sweden

    Henrik Green

  5. Division of Drug Research, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden

    Henrik Green

  6. Department of Clinical Genetics, Department of Biomedical and Clinical Sciences, Centre for Rare Diseases in South East Region of Sweden, Linköping University, Linköping, Sweden

    Cecilia Gunnarsson

  7. Pediatric Heart Centre, Skåne University Hospital and Department of Clinical Sciences, Lund University, Lund, Sweden

    Petru Liuba & Eva Fernlund

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  1. Anna Wålinder Österberg
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Contributions

All authors contributed to the study conception and design. Material preparation and data collection were performed by AWÖ, RJ, MC, and EF. Analysis was performed by AWÖ with support from all other authors. The first draft of the manuscript was written by AWÖ and all authors commented on previous versions of the manuscript. All authors have read and approved the final manuscript. All the above authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

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Correspondence to Eva Fernlund.

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Ethical Approval

The study was approved by the Regional Ethical Review Board in Lund, Sweden, H15 2009/616 and 2011/668. The study conforms to the ethical guidelines of the 1975 Declaration of Helsinki.

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An informed consent was obtained from all participants or their legal guardians (for participants < 18 years of age).

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Österberg, A.W., Östman-Smith, I., Jablonowski, R. et al. High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young. Pediatr Cardiol 42, 492–500 (2021). https://doi.org/10.1007/s00246-020-02506-9

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