Document Detail


Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance.
MedLine Citation:
PMID:  19589434     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Martin S Maron; 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
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  54     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-10     Completed Date:  2009-07-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  220-8     Citation Subset:  AIM; IM    
Affiliation:
Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts 02111, USA. mmaron@tuftsmedialcenter.org
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Cardiomyopathy, Hypertrophic / diagnosis*,  genetics,  radionuclide imaging,  ultrasonography
Child
Echocardiography
Female
Fibrosis
Gadolinium / diagnostic use
Humans
Hypertrophy, Left Ventricular / diagnosis*,  genetics,  radionuclide imaging,  ultrasonography
Magnetic Resonance Imaging*
Male
Middle Aged
Phenotype
Prospective Studies
Time Factors
Ventricular Outflow Obstruction / diagnosis,  genetics
Young Adult
Chemical
Reg. No./Substance:
7440-54-2/Gadolinium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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