RIGHT-VENTRICULAR BIOPSY FINDINGS OF HYPERTROPHIC CARDIOMYOPATHY IN APATIENT WITH NOONAN-SYNDROME

Authors
SKINKER DM COTTRILL CM OCONNOR WN
Citation
Dm. Skinker et al., RIGHT-VENTRICULAR BIOPSY FINDINGS OF HYPERTROPHIC CARDIOMYOPATHY IN APATIENT WITH NOONAN-SYNDROME, Cardiology in the young, 8(2), 1998, pp. 256-259
Citations number
10
Categorie Soggetti
Pediatrics,"Cardiac & Cardiovascular System
Journal title
Cardiology in the young → ACNP
ISSN journal
10479511
Volume
8
Issue
2
Year of publication
1998
Pages
256 - 259
Database
ISI
SICI code
1047-9511(1998)8:2<256:RBFOHC>2.0.ZU;2-X
Abstract
We report right ventricular ultrastructure in a 20-year-old patient wi th Noonan's Syndrome. Initial left ventricular and septal myocardial h ypertrophy, observed at 2 months without obstruction, progressed to bi ventricular involvement over 18 years. Histopathologic evidence of car diomyocytic disarray, with ultrastructural evidence of myofibrillar di sarray, confirmed typical biopsy findings of hypertrophic cardiomyopat hy on the right ventricular side of the septum.