Cardiovascular magnetic resonance predictors of heart failure in hypertrophic cardiomyopathy: the role of myocardial replacement fibrosis and the microcirculation

Claire E. Raphael, Frances M. Mitchell, Gajen Sunthar Kanaganayagam, Alphonsus C. Liew, Elisa Di Pietro, Miguel Silva Vieira, Lina Kanapeckaite, Simon J. Newsome, John Gregson, Ruth Owen, Li Yueh Hsu, Vassilios Vassiliou, Robert Cooper, Aamir Ali, Tevfik F. Ismail, Brandon Wong , Kristi Sun , Peter D. Gatehouse, David Firmin, Stuart A. CookMichael Frenneaux, Andrew Arai, Rory O’Hanlon, Dudley J. Pennell, Sanjay K. Prasad

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Abstract

Introduction: Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality. Predictors of HF, in particular the role of myocardial fibrosis and microvascular ischemia remain unclear. We assessed the predictive value of cardiovascular magnetic resonance (CMR) for development of HF in HCM in an observational cohort study.

Methods: Serial patients with HCM underwent CMR, including adenosine first-pass perfusion, left atrial (LA) and left ventricular (LV) volumes indexed to body surface area (i) and late gadolinium enhancement (%LGE- as a % of total myocardial mass). We used a composite endpoint of HF death, cardiac transplantation, and progression to NYHA class III/IV.

Results: A total of 543 patients with HCM underwent CMR, of whom 94 met the composite endpoint at baseline. The remaining 449 patients were followed for a median of 5.6 years. Thirty nine patients (8.7%) reached the composite endpoint of HF death (n = 7), cardiac transplantation (n = 2) and progression to NYHA class III/IV (n = 20). The annual incidence of HF was 2.0 per 100 person-years, 95% CI (1.6–2.6). Age, previous non-sustained ventricular tachycardia, LV end-systolic volume indexed to body surface area (LVESVI), LA volume index ; LV ejection fraction, %LGE and presence of mitral regurgitation were significant univariable predictors of HF, with LVESVI (Hazard ratio (HR) 1.44, 95% confidence interval (95% CI) 1.16–1.78, p = 0.001), %LGE per 10% (HR 1.44, 95%CI 1.14–1.82, p = 0.002) age (HR 1.37, 95% CI 1.06–1.77, p = 0.02) and mitral regurgitation (HR 2.6, p = 0.02) remaining independently predictive on multivariable analysis. The presence or extent of inducible perfusion defect assessed using a visual score did not predict outcome (p = 0.16, p = 0.27 respectively).

Discussion: The annual incidence of HF in a contemporary ambulatory HCM population undergoing CMR is low. Myocardial fibrosis and LVESVI are strongly predictive of future HF, however CMR visual assessment of myocardial perfusion was not.
Original languageEnglish
Article number26
JournalJournal of Cardiovascular Magnetic Resonance
Volume23
DOIs
Publication statusPublished - 8 Mar 2021

Keywords

  • Cardiovascular magnetic resonance
  • Fibrosis
  • Heart failure
  • Hypertrophic cardiomyopathy
  • Microvascular ischemia
  • Myocardial perfusion
  • Prognosis

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