Journal article
Progression of myocardial fibrosis in hypertrophic cardiomyopathy: mechanisms and clinical implications
- Abstract:
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Aims Myocardial fibrosis as detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) is a powerful prognostic marker in hypertrophic cardiomyopathy (HCM) and may be progressive. The precise mechanisms underlying fibrosis progression are unclear. We sought to assess the extent of LGE progression in HCM and explore potential causal mechanisms and clinical implications.
Methods and results Seventy-two HCM patients had two CMR (CMR1-CMR2) at an interval of 5.7 ± 2.8 years with annual clinical follow-up for 6.3 ± 3.6 years from CMR1. A combined endpoint of heart failure progression, cardiac hospitalization, and new onset ventricular tachycardia was assessed. Cine and LGE imaging were performed to assess left ventricular (LV) mass, function, and fibrosis on serial CMR. Stress perfusion imaging and cardiac energetics were undertaken in 38 patients on baseline CMR (CMR1). LGE mass increased from median 4.98 g [interquartile range (IQR) 0.97–13.48 g] to 6.30 g (IQR 1.38–17.51 g) from CMR1 to CMR2. Substantial LGE progression (ΔLGE ≥ 4.75 g) occurred in 26% of patients. LGE increment was significantly higher in those with impaired myocardial perfusion reserve (≺MPRI 1.40) and energetics (phosphocreatine/adenosine triphosphate ≺1.44) on baseline CMR (P ≤ 0.01 for both). Substantial LGE progression was associated with LV thinning, increased cavity size and reduced systolic function, and conferred a five-fold increased risk of subsequent clinical events (hazard ratio 5.04, 95% confidence interval 1.85–13.79; P = 0.002).
Conclusion Myocardial fibrosis is progressive in some HCM patients. Impaired energetics and perfusion abnormalities are possible mechanistic drivers of the fibrotic process. Fibrosis progression is associated with adverse cardiac remodelling and predicts an increased risk of subsequent clinical events in HCM.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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(Preview, Version of record, pdf, 842.9KB, Terms of use)
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- Publisher copy:
- 10.1093/ehjci/jey135
Authors
- Funding agency for:
- Ariga, R
- Lewandowski, AJ
- Grant:
- Clinical Research Training Fellowship: 098436/Z/12/Z
- FS/18/3/33292
- Funding agency for:
- Watkins, H
- Neubauer, S
- Grant:
- U01HL117006-01A1
- U01HL117006-01A1
- Funding agency for:
- Watkins, H
- Neubauer, S
- Grant:
- U01HL117006-01A1
- U01HL117006-01A1
- Funding agency for:
- Rodgers, C
- Grant:
- Sir Henry Dale Fellowship: 098436/Z/12/B
- Funding agency for:
- Raman, B
- Mahmod, M
- Spartera, M
- Chan, K
- Grant:
- Oxford Biomedical Research Centre
- Oxford Biomedical Research Centre
- Academic Clinical Fellowship
- Publisher:
- Oxford University Press
- Journal:
- European Heart Journal: Cardiovascular Imaging More from this journal
- Volume:
- 20
- Issue:
- 2
- Pages:
- 157–167
- Publication date:
- 2018-10-24
- Acceptance date:
- 2018-09-05
- DOI:
- EISSN:
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2047-2412
- ISSN:
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2047-2404
- Keywords:
- Pubs id:
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pubs:935936
- UUID:
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uuid:c26a77fc-9c75-4d9b-8c4d-0ce1eb3c5696
- Local pid:
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pubs:935936
- Deposit date:
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2018-10-31
Terms of use
- Copyright holder:
- Raman et al
- Copyright date:
- 2018
- Notes:
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The Author(s) 2019. Published by Oxford University Press on behalf of the European Society of Cardiology.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
- Licence:
- CC Attribution (CC BY)
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