Cardiovascular Magnetic Resonance Predictors of Heart Failure in Hypertrophic Cardiomyopathy: The Role of Myocardial Replacement Fibrosis and the Microcirculation

Claire E. Raphael, Frances Mitchell, Gajen Kanaganayagam, Alphonsus Liew, Elisa Di Pietro, Miguel Vieira, Lina Kanapeckaite, Simon Newsome, John Gregson, Ruth Owen, Li-Yueh Hsu, Vassilis Vassiliou, Robert Cooper, Aamir Ali, Tevfik Ismail, Brandon Wong, Kristi Sun, Peter D. Gatehouse, David Firmin, Stuart A. CookMichael P. Frenneaux, Andrew E. 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 heart failure, 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 (LAV) 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, cardiac transplantation and progression to NYHA class III/IV. 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), LAVi, 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 was low. Myocardial fibrosis and LV end systolic volume were strongly predictive of future HF, however CMR visual assessment of myocardial perfusion was not.
Original languageEnglish
JournalJournal of cardiovascular magnetic resonance
Publication statusAccepted/In press - 1 Feb 2021

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