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Right ventricular biopsy findings of hypertrophic cardiomyopathy in a patient with Noonan syndrome

Published online by Cambridge University Press:  19 August 2008

Donna M. Skinker ,
Carol M. Cottrill  and
William N. O'Connor
Donna M. Skinker
Affiliation:
From the Departments of Pathology and Pediatrics, University of Kentucky, Chandler Medical Center, Lexington, Kentucky, USA
Carol M. Cottrill
Affiliation:
From the Departments of Pathology and Pediatrics, University of Kentucky, Chandler Medical Center, Lexington, Kentucky, USA
William N. O'Connor*
Affiliation:
From the Departments of Pathology and Pediatrics, University of Kentucky, Chandler Medical Center, Lexington, Kentucky, USA
*
Dr. W. N. O'Connor, Department of Pathology and Laboratory Medicine, University of Kentucky, Chandler Medical Center, 800 Rose Street, Lexington, Kentucky 40536, USA. Tel: (606) 257-5357; Fax: (606) 323-2094
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Summary

We report right ventricular ultrastructure in a 20-year-old patient with Noonan's Syndrome. Initial left ventricular and septal myocardial hypertrophy, observed at 2 months without obstruction, progressed to biventricular involvement over 18 years. Histopathologic evidence of cardiomyocytic disarray, with ultrastructural evidence of myofibrillar disarray, confirmed typical biopsy findings of hypertrophic cardiomyopathy on the right ventricular side of the septum.

Keywords

Hypertrophic cardiomyoparhyNoonan syndromecardiomyocyte disarray
Type
Brief Reports
Information
Cardiology in the Young , Volume 8 , Issue 2 , April 1998 , pp. 256 - 259
Copyright
Copyright © Cambridge University Press 1998

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