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Arrhythmogenic cardiomyopathy and differential diagnosis with physiological right ventricular remodelling in athletes using cardiovascular magnetic resonance

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Abstract

To describe the overlap between structural abnormalities typical of arrhythmogenic right ventricular cardiomyopathy (ARVC) and physiological right ventricular adaptation to exercise and differentiate between pathologic and physiologic findings using CMR. We compared CMR studies of 43 patients (mean age 49 ± 17 years, 49% males, 32 genotyped) with a definitive diagnosis of ARVC with 97 (mean age 45 ± 16 years, 61% males) healthy athletes. CMR was abnormal in 37 (86%) patients with ARVC, but only 23 (53%) fulfilled a major or minor CMR criterion according to the TFC. 7/20 patients who did not fulfil any CMR TFC showed pathological finding (RV RWMA and fibrosis in the LV or LV RWMA). RV was affected in isolation in 17 (39%) patients and 18 (42%) patients showed biventricular involvement. Common RV abnormalities included RWMA (n = 34; 79%), RV dilatation (n = 18; 42%), RV systolic dysfunction (≤ 45%) (n = 17; 40%) and RV LGE (n = 13; 30%). The predominant LV abnormality was LGE (n = 20; 47%). 22/32 (69%) patients exhibited a pathogenic variant: PKP2 (n = 17, 53%), DSP (n = 4, 13%) and DSC2 (n = 1, 3%). Sixteen (16%) athletes exceeded TFC cut-off values for RV volumes. None of the athletes exceeded a RV/LV end-diastolic volume ratio > 1.2, nor fulfilled TFC for impaired RV ejection fraction. The majority (86%) of ARVC patients demonstrate CMR abnormalities suggestive of cardiomyopathy but only 53% fulfil at least one of the CMR TFC. LV involvement is found in 50% cases. In athletes, an RV/LV end-diastolic volume ratio > 1.2 and impaired RV function (RVEF ≤ 45%) are strong predictors of pathology.

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Acknowledgements

Cardiac risk in the young (CRY).

Funding

Gherardo Finocchiaro, Micheal Papadakis and Sanjay Sharma have received research grants from Cardiac Risk in the Young (CRY). Gherardo Finocchiaro has received a research grant from the Charles Wolfson Charitable Trust.

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Author notes

  1. Gherardo Finocchiaro, Michael Papadakis equally contributed as senior authors.

Authors and Affiliations

  1. Cardiology Clinical Academic Group, St George’s University of London and St George’s University Hospital NHS Foundation Trust, London, UK

    Eleonora Moccia, Efstathios Papatheodorou, Chris J. Miles, Ahmed Merghani, Aneil Malhotra, Harshil Dhutia, Rachel Bastiaenen, Nabeel Sheikh, Abbas Zaidi, Tessa Homfray, Nicholas Bunce, Lisa J. Anderson, Maite Tome, Elijah Behr, Sanjay Sharma, Gherardo Finocchiaro & Michael Papadakis

  2. Clinical and Interventional Cardiology, Sassari University Hospital, Via Enrico de Nicola 1, Sassari, Italy

    Eleonora Moccia & Giuseppe Damiano Sanna

  3. Guy’s and St, Thomas’s Hospital, London, UK

    Rachel Bastiaenen, Nabeel Sheikh & Gherardo Finocchiaro

  4. Barts Heart Centre, St Bartholomew’s Hospital, London, UK

    James Moon

  5. Institute of Cardiovascular Science, University College London, London, UK

    James Moon

  6. University Hospital of Wales, Cardiology, Cardiff, UK

    Abbas Zaidi

Authors
  1. Eleonora Moccia
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by EM, EP and GF. The first draft of the manuscript was written by EM, GF and MP; all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Eleonora Moccia.

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The authors declare no competing interests.

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The authors have no relevant financial or non-financial interests to disclose.

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This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the National Research Ethics Service and the Southwest-Central Bristol committee.

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Moccia, E., Papatheodorou, E., Miles, C.J. et al. Arrhythmogenic cardiomyopathy and differential diagnosis with physiological right ventricular remodelling in athletes using cardiovascular magnetic resonance. Int J Cardiovasc Imaging 38, 2723–2732 (2022). https://doi.org/10.1007/s10554-022-02684-y

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