Published Online
on June 23, 2008

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Original Article

Clinical Profile and Significance of Delayed Enhancement in Hypertrophic Cardiomyopathy

Martin Maron^1,6; Evan Appelbaum²; Caitlin Harrigan³; Jacki Buros⁴; C. Michael Gibson²; Connie Hanna⁵; John R. Lesser⁵; James E. Udelson¹; Warren J. Manning² and Barry J. Maron⁵

¹ Tufts-New England Medical Center, Boston, Massachusetts;
² Beth Israel Deaconess Medical Center; PERFUSE Core Laboratory, Boston, Massachusetts;
³ PEPRFUSE Core Laboratory, Boston, Massachusetts;
⁴ Perfuse Core Laboratory, Boston, Massachusetts;
⁵ Minneapolis Heart Institute Foundation, Minneapolis, Minnesota

⁶ E-mail: mmaron{at}tufts-nemc.org

Background—Contrast-enhanced cardiovascular magnetic resonance (CMR) with delayed enhancement (DE) can provide in vivo assessment of myocardial fibrosis. However, the clinical significance of DE in hypertrophic cardiomyopathy (HCM) remains unresolved.

Methods and Results—Cine and CMR-DE were performed in 202 HCM patients (42±17 years; 71% male) and DE was compared to clinical and demographic variables and patients were followed up for 681±249 days for adverse disease events. DE was identified in 111 (55%) HCM patients, occupying 9±11% of LV myocardial volume, including >25% DE in 10% of patients. Presence of DE was related to occurrence of heart failure symptoms (p=0.05) and LV systolic dysfunction (p=0.001). DE was present in all patients with ejection fraction (EF) 50%, but also in 53% (102/192) of patients with preserved EF (p<0.001); %DE was both inversely related to (r= -0.3; p<0.001) and an independent predictor of EF (r= -0.4; p<0.001). Delayed enhancement (7±7% of LV) was present in 54 patients who were asymptomatic (and with normal EF). Over the follow-up period, the annualized adverse cardiovascular event rate in patients with DE exceeded that in patients without DE, but did not achieve statistical significance (5.5 % vs. 3.3 %; p=0.5).

Conclusions—In a large HCM cohort, DE was an independent predictor of systolic dysfunction but with only a modest relationship to heart failure symptoms. These data suggest an important role for myocardial fibrosis in the clinical course of HCM patients, but are not sufficient at this time to consider DE as an independent risk factor for adverse prognosis.

Key Words: heart failure • magnetic resonance imaging • fibrosis • hypertrophic cardiomyopathy