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FOCUS ISSUE: HYPERTROPHIC CARDIOMYOPATHY: CLINICAL RESEARCH

Hypertrophic Cardiomyopathy Phenotype Revisited After 50 Years With Cardiovascular Magnetic Resonance

Martin S. Maron, MD^_*,_*, Barry J. Maron, MD, Caitlin Harrigan, BA^_*, Jacki Buros, BA, C. Michael Gibson, MD, MS^,, Iacopo Olivotto, MD^||, Leah Biller, BA, John R. Lesser, MD, James E. Udelson, MD^_*, Warren J. Manning, MD^, and Evan Appelbaum, MD^,

^_* Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts
The Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota
PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, Massachusetts
Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
^|| Regional Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy

Manuscript received February 13, 2009; revised manuscript received April 30, 2009, accepted May 5, 2009.

^_* Reprint requests and correspondence: Dr. Martin S. Maron, Tufts Medical Center, #70, 800 Washington Street, Boston, Massachusetts 02111 (Email: mmaron{at}tuftsmedialcenter.org).

Objectives: Our purpose was to characterize the pattern and distribution of left ventricular (LV) hypertrophy by cardiovascular magnetic resonance (CMR) to more precisely define phenotypic expression and its clinical implications in hypertrophic cardiomyopathy (HCM).

Background: Based on prior pathologic and 2-dimensional echocardiographic studies, HCM has been regarded as a disease characterized by substantial LV wall thickening.

Methods: Cine and late gadolinium enhancement CMR were performed in 333 consecutive HCM patients (age 43 ± 17 years).

Results: Basal anterior LV free wall and the contiguous anterior ventricular septum were the most commonly hypertrophied segments (n = 256; 77%). LV hypertrophy was focal (involving 2 segments [12% of LV]) in 41 patients (12%), intermediate (3 to 7 segments [13% to 49% of LV]) in 112 patients (34%), and diffuse (8 segments [50% of LV]) in 180 patients (54%); 42 patients (13%) showed hypertrophied segments separated by regions of normal thickness. The number of hypertrophied segments was greater in patients with LV outflow tract obstruction (30 mm Hg) than without (10 ± 4 vs. 8 ± 4 per patient; p = 0.0001) and was associated with an advanced New York Heart Association functional class (p = 0.007). LV wall thickness was greater in segments with late gadolinium enhancement than without (20 ± 6 mm vs. 16 ± 6 mm; p < 0.001). We also identified 40 (12%) of HCM patients with segmental LV hypertrophy largely confined to the anterolateral free wall, posterior septum, or apex, which was underestimated or undetected by echocardiography.

Conclusions: Although diverse, patterns of LV hypertrophy are usually not extensive in HCM, involving 50% of the chamber in about one-half the patients, and are particularly limited in extent in an important minority. Contiguous portions of anterior free wall and septum constituted the predominant region of wall thickening, with implications for clinical diagnosis. These observations support an emerging role for CMR in the contemporary evaluation of patients with HCM.

Key Words: hypertrophic cardiomyopathy • hypertrophy • magnetic resonance imaging • fibrosis

Abbreviations and Acronyms   CMR = cardiovascular magnetic resonance   HCM = hypertrophic cardiomyopathy   LGE = late gadolinium enhancement   LV = left ventricle/ventricular   NYHA = New York Heart Association

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