Abstract
IntroductionHeart 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.MethodsSerial 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.ResultsA 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).DiscussionThe 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.
Highlights
Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality
Patient recruitment Consecutive patients with a diagnosis of HCM seen in the inherited cardiomyopathy service or referred to the Royal Brompton Hospital for cardiovascular magnetic resonance (CMR) between December 2003 and April 2013 were prospectively recruited into a registry
There was no significant correlation between the severity of perfusion defect and the %Late gadolinium enhancement (LGE) (r = 0.05, p = 0.34), patients without a perfusion defect had a lower %LGE compared to those with a perfusion defect (median LGE 7.9% (IQR 1.1– 16.3) vs 13.1% (3.8–24.7, p = 0.04)
Summary
Heart failure (HF) in hypertrophic cardiomyopathy (HCM) is associated with high morbidity and mortality. Patients with hypertrophic cardiomyopathy (HCM) are at risk of heart failure (HF) [1,2,3] and the annual mortality in these patients is ten-fold higher than the general HCM population. Patients with HCM and HF have a high risk of death from both progressive pump failure and sudden cardiac death (SCD) [4, 5]. Patients with HCM often have abnormal myocardial perfusion [6] and recurrent bouts of ischaemia are hypothesised to lead to myocardial fibrosis and development of systolic dysfunction [7, 8]. Replacement myocardial fibrosis has been shown to predict SCD in HCM [9, 10] but its relationship to HF is not clear
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