Document Detail

T1 measurements identify extracellular volume expansion in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy.
MedLine Citation:
PMID:  23549607     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM) and a potential substrate for arrhythmias and heart failure. Sarcomere mutations seem to induce profibrotic changes before left ventricular hypertrophy (LVH) develops. To further evaluate these processes, we used cardiac magnetic resonance with T1 measurements on a genotyped HCM population to quantify myocardial extracellular volume (ECV).
METHODS AND RESULTS: Sarcomere mutation carriers with LVH (G+/LVH+, n=37) and without LVH (G+/LVH-, n=29), patients with HCM without mutations (sarcomere-negative HCM, n=11), and healthy controls (n=11) underwent contrast cardiac magnetic resonance, measuring T1 times pre- and postgadolinium infusion. Concurrent echocardiography and serum biomarkers of collagen synthesis, hemodynamic stress, and myocardial injury were also available in a subset. Compared with controls, ECV was increased in patients with overt HCM, as well as G+/LVH- mutation carriers (ECV=0.36±0.01, 0.33±0.01, 0.27±0.01 in G+/LVH+, G+/LVH-, controls, respectively; P≤0.001 for all comparisons). ECV correlated with N-terminal probrain natriuretic peptide levels (r=0.58; P<0.001) and global E' velocity (r=-0.48; P<0.001). Late gadolinium enhancement was present in >60% of overt patients with HCM but absent from G+/LVH- subjects. Both ECV and late gadolinium enhancement were more extensive in sarcomeric HCM than sarcomere-negative HCM.
CONCLUSIONS: Myocardial ECV is increased in HCM sarcomere mutation carriers even in the absence of LVH. These data provide additional support that fibrotic remodeling is triggered early in disease pathogenesis. Quantifying ECV may help characterize the development of myocardial fibrosis in HCM and ultimately assist in developing novel disease-modifying therapy, targeting interstitial fibrosis.
Carolyn Y Ho; Siddique A Abbasi; Tomas G Neilan; Ravi V Shah; Yucheng Chen; Bobak Heydari; Allison L Cirino; Neal K Lakdawala; E John Orav; Arantxa González; Begoña López; Javier Díez; Michael Jerosch-Herold; Raymond Y Kwong
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2013-04-02
Journal Detail:
Title:  Circulation. Cardiovascular imaging     Volume:  6     ISSN:  1942-0080     ISO Abbreviation:  Circ Cardiovasc Imaging     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-05-22     Completed Date:  2013-07-18     Revised Date:  2014-05-07    
Medline Journal Info:
Nlm Unique ID:  101479935     Medline TA:  Circ Cardiovasc Imaging     Country:  United States    
Other Details:
Languages:  eng     Pagination:  415-22     Citation Subset:  IM    
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MeSH Terms
Analysis of Variance
Biological Markers / blood
Cardiomyopathy, Hypertrophic / blood,  diagnosis*,  genetics,  pathology,  physiopathology
Case-Control Studies
Collagen / blood
Contrast Media / diagnostic use
DNA Mutational Analysis
Disease Progression
Extracellular Matrix / metabolism,  pathology*
Gadolinium DTPA / diagnostic use
Genetic Predisposition to Disease
Hypertrophy, Left Ventricular / blood,  diagnosis*,  genetics,  pathology,  physiopathology
Logistic Models
Magnetic Resonance Imaging, Cine*
Middle Aged
Myocardium / metabolism,  pathology*
Predictive Value of Tests
Sarcomeres / metabolism,  pathology*
Ventricular Remodeling* / genetics
Young Adult
Grant Support
Reg. No./Substance:
0/Biological Markers; 0/Contrast Media; 9007-34-5/Collagen; K2I13DR72L/Gadolinium DTPA

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

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