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FOCUS ISSUE: HYPERTROPHIC CARDIOMYOPATHY: CLINICAL RESEARCH

Cardiac Magnetic Resonance Detection of Myocardial Scarring in Hypertrophic Cardiomyopathy

Correlation With Histopathology and Prevalence of Ventricular Tachycardia

Deborah H. Kwon, MD^_*, Nicholas G. Smedira, MD^_*, E. Rene Rodriguez, MD, Carmela Tan, MD, Randolph Setser, PhD, Maran Thamilarasan, MD^_*, Bruce W. Lytle, MD^_*, Harry M. Lever, MD^_* and Milind Y. Desai, MD^_*,,_*

^_* Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio
Department of Pathology, Cleveland Clinic, Cleveland, Ohio
Imaging Institute, Cleveland Clinic, Cleveland, Ohio

Manuscript received December 27, 2008; revised manuscript received February 23, 2009, accepted April 3, 2009.

^_* Reprint requests and correspondence: Dr. Milind Y. Desai, Director, Cardiac CT and MR, Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44195 (Email: desaim2{at}ccf.org).

Objectives: In hypertrophic cardiomyopathy (HCM) patients undergoing surgical myectomy, we sought to determine the association between pre-operative cardiac magnetic resonance (CMR) findings, small intramural coronary arteriole dysplasia (SICAD) on histopathology, and ventricular tachycardia (VT).

Background: Myocardial scarring (fibrosis) and SICAD are frequently observed on histopathology in HCM patients. CMR measures wall thickness and detects scar.

Methods: Sixty symptomatic HCM patients (62% men; mean age 51 ± 14 years), with preserved ejection fraction (mean 64 ± 5%) and no angiographic coronary disease underwent CMR (cine and delayed post-contrast) using a Siemens 1.5 T scanner, followed by septal myectomy. Maximal basal septal thickness was recorded on cine CMR. Scar was determined (percentage of total myocardium) on delayed post-contrast CMR images and quantified as none, mild (0% to 25%), moderate (26% to 50%), or severe (>50%). VT was assessed using Holter monitoring. Degree of SICAD was determined (normal, mild, moderate, and severe) on histopathology of surgical specimen.

Results: SICAD and scar were seen in 45 (75%) and 38 (63%) patients, respectively. In 15 patients without SICAD, 12 (80%) had no scar; 23 (70%) patients with mild SICAD had mild scar on CMR. On multivariate analysis, degree of SICAD was independently associated with scar on CMR (Wald chi-square statistic: 6.8, p < 0.01). Patients with basal septal scar on CMR had higher VT frequency compared with those without (27% vs. 5%, p = 0.03).

Conclusions: A strong association exists between degree of SICAD and myocardial scarring seen on CMR.

Key Words: hypertrophic cardiomyopathy • small intramural coronary arteriole dysplasia • myocardial scarring • cardiac magnetic resonance

Abbreviations and Acronyms   CAD = coronary artery disease   CI = confidence interval   CMR = cardiac magnetic resonance   DHE-MRI = delayed hyperenhancement magnetic resonance imaging   HCM = hypertrophic cardiomyopathy   LV = left ventricle/ventricular   LVEF = left ventricular ejection fraction   LVOT = left ventricular outflow tract   ROC = receiver-operating characteristic   SCD = sudden cardiac death   SICAD = small intramural coronary arteriole dysplasia   VT = ventricular tachycardia

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