Literature DB >> 19589434

Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance.

Martin S Maron1, Barry J Maron, Caitlin Harrigan, Jacki Buros, C Michael Gibson, Iacopo Olivotto, Leah Biller, John R Lesser, James E Udelson, Warren J Manning, Evan Appelbaum.   

Abstract

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 < or = 2 segments [< or = 12% of LV]) in 41 patients (12%), intermediate (3 to 7 segments [13% to 49% of LV]) in 112 patients (34%), and diffuse (> or = 8 segments [> or = 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 (> or = 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 < or = 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.

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Year:  2009        PMID: 19589434     DOI: 10.1016/j.jacc.2009.05.006

Source DB:  PubMed          Journal:  J Am Coll Cardiol        ISSN: 0735-1097            Impact factor:   24.094


  97 in total

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2.  Cardiac MRI assessed left ventricular hypertrophy in differentiating hypertensive heart disease from hypertrophic cardiomyopathy attributable to a sarcomeric gene mutation.

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Review 8.  Cardiovascular magnetic resonance: applications in daily practice.

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9.  Assessment of myocardial fibrosis and coronary arteries in hypertrophic cardiomyopathy using combined arterial and delayed enhanced CT: comparison with MR and coronary angiography.

Authors:  Lei Zhao; Xiaohai Ma; Mark C Delano; Tengyong Jiang; Chen Zhang; Yi Liu; Zhaoqi Zhang
Journal:  Eur Radiol       Date:  2012-10-14       Impact factor: 5.315

10.  Hypertrophic cardiomyopathy in the Saudi Arabian population: Clinical and echocardiographic characteristics and outcome analysis.

Authors:  Waqas Ahmed; Naveed Akhtar; Odd Bech-Hanssen; Bader Al Mahdi; Talal Al Otaibi; Bahaa M Fadel
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