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J Am Soc Echocardiogr. 2009 Dec;22(12):1327-34. doi: 10.1016/j.echo.2009.08.014. Epub 2009 Oct 7.

Diastolic dysfunction and its histopathological correlation in obstructive hypertrophic cardiomyopathy in children and adolescents.

Author information

1
Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA. shaji.menon@utah.edu

Erratum in

  • J Am Soc Echocardiogr. 2010 Feb;23(2):222.

Abstract

BACKGROUND:

Histopathologic hallmarks of hypertrophic cardiomyopathy (HCM) include myocyte hypertrophy and disarray as well as interstitial and endocardial fibrosis. Published correlations between echocardiographic parameters and histopathologic findings are scarce.

METHODS:

All patients aged <20 years (n = 45; 15 female patients; median age, 14 years) with obstructive HCM undergoing septal myectomy at the Mayo Clinic from 2003 to 2007 were identified. A retrospective review of echocardiographic data was performed, and these data were compared with the histologic findings from the myectomy specimens.

RESULTS:

Histopathologic analysis of myectomy specimens revealed significant myocyte hypertrophy (100%), myocyte disarray (98%), interstitial fibrosis (95%), and subendocardial fibrosis (97%). On multivariate regression analysis, there was a significant relationship between the degree of myocyte disarray and echocardiographic markers of left ventricular diastolic dysfunction.

CONCLUSION:

The results of this study suggest that myocyte disarray is a key factor responsible for diastolic dysfunction in pediatric patients with obstructive HCM. These findings provide novel insights into the mechanism of diastolic dysfunction in HCM that warrant further study.

PMID:
19815379
DOI:
10.1016/j.echo.2009.08.014
[Indexed for MEDLINE]

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