Clinical Research
Hypertrophic Cardiomyopathy
Prognostic Significance of Myocardial Fibrosis in Hypertrophic Cardiomyopathy

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Objectives

We investigated the significance of fibrosis detected by late gadolinium enhancement cardiovascular magnetic resonance for the prediction of major clinical events in hypertrophic cardiomyopathy (HCM).

Background

The role of myocardial fibrosis in the prediction of sudden death and heart failure in HCM is unclear with a lack of prospective data.

Methods

We assessed the presence and amount of myocardial fibrosis in HCM patients and prospectively followed them for the development of morbidity and mortality in patients over 3.1 ± 1.7 years.

Results

Of 217 consecutive HCM patients, 136 (63%) showed fibrosis. Thirty-four of the 136 patients (25%) in the fibrosis group but only 6 of 81 (7.4%) patients without fibrosis reached the combined primary end point of cardiovascular death, unplanned cardiovascular admission, sustained ventricular tachycardia or ventricular fibrillation, or appropriate implantable cardioverter-defibrillator discharge (hazard ratio [HR]: 3.4, p = 0.006). In the fibrosis group, overall risk increased with the extent of fibrosis (HR: 1.18/5% increase, p = 0.008). The risk of unplanned heart failure admissions, deterioration to New York Heart Association functional class III or IV, or heart failure-related death was greater in the fibrosis group (HR: 2.5, p = 0.021), and this risk increased as the extent of fibrosis increased (HR: 1.16/5% increase, p = 0.017). All relationships remained significant after multivariate analysis. The extent of fibrosis and nonsustained ventricular tachycardia were univariate predictors for arrhythmic end points (sustained ventricular tachycardia or ventricular fibrillation, appropriate implantable cardioverter-defibrillator discharge, sudden cardiac death) (HR: 1.30, p = 0.014). Nonsustained ventricular tachycardia remained an independent predictor of arrhythmic end points after multivariate analysis, but the extent of fibrosis did not.

Conclusions

In patients with HCM, myocardial fibrosis as measured by late gadolinium enhancement cardiovascular magnetic resonance is an independent predictor of adverse outcome. (The Prognostic Significance of Fibrosis Detection in Cardiomyopathy; NCT00930735)

Key Words

cardiovascular magnetic resonance
hypertrophic cardiomyopathy
late gadolinium enhancement
myocardial fibrosis

Abbreviations and Acronyms

BSA
body surface area
CI
confidence interval
CMR
cardiovascular magnetic resonance
FWHM
full width half maximum
HCM
hypertrophic cardiomyopathy
HF
heart failure
HR
hazard ratio
ICD
implantable cardioverter-defibrillator
LAVi
left atrial volume index
LGE
late gadolinium enhancement
LVOTO
left ventricular outflow tract obstruction
NSVT
nonsustained ventricular tachycardia
NYHA
New York Heart Association
SCD
sudden cardiac death

Cited by (0)

Research support was received from the National Institutes of Health Cardiovascular Biomedical Research Unit, British Heart Foundation, and (a registered charity). Dr. O'Hanlon was supported by the Department of Health, Ireland. Dr. Cowie has consultancy agreements with Pfizer, Takeda, and Servier. Dr. Pennell has received consultant/research support from Siemens, and is a director for Cardiovascular Imaging Solutions. All other authors have reported that they have no relationships to disclose.