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Original Articles

Clinical Profile and Significance of Delayed Enhancement in Hypertrophic Cardiomyopathy

Martin S. Maron, MD; Evan Appelbaum, MD; Caitlin J. Harrigan, BA; Jacki Buros, BA; C. Michael Gibson, MD, MS; Connie Hanna, RN; John R. Lesser, MD; James E. Udelson, MD; Warren J. Manning, MD and Barry J. Maron, MD

From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Mass (M.S.M., J.E.U.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Mass (E.A., C.M.G., W.J.M.); PERFUSE Angiographic Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, Mass (E.A., C.J.H., J.B., C.M.G., W.J.M.); and the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minn (C.H., J.R.L., B.J.M.).

Correspondence to Martin S. Maron, MD, Tufts Medical Center, #70, 800 Washington Street, Boston, Massachusetts 02111. E-mail mmaron{at}tuftsmedicalcenter.org

Received January 22, 2008; accepted May 23, 2008.

Background— Contrast-enhanced cardiovascular magnetic resonance 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 cardiovascular magnetic resonance with DE were performed in 202 HCM patients (mean age, 42±17 years; 71% male), DE was compared with 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 left ventricular myocardial volume, including >25% DE in 10% of patients. The presence of DE was related to occurrence of heart failure symptoms (P=0.05) and left ventricular systolic dysfunction (P=0.001). DE was present in all patients with ejection fraction 50% but also in 53% (102/192) of patients with preserved ejection fraction (P<0.001); %DE was both inversely related to (r=–0.3; P<0.001) and an independent predictor of ejection fraction (r=–0.4; P<0.001). DE (7%±7% of left ventricle) was present in 54 patients who were asymptomatic (and with normal ejection fraction). 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% versus 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 • MRI • fibrosis • hypertrophic cardiomyopathy

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CLINICAL PERSPECTIVE