title: Myocardial fragmentation associated with disruption of the Z-band in hypertrophic cardiomyopathy in Noonan syndrome.
abstract: A 13-year-old female with Noonan syndrome had been diagnosed with hypertrophic cardiomyopathy, and she died of heart failure at the age of 25 years. Light microscopic and electron microscopic examination of her biopsied myocardium and autopsy heart showed myocardial fragmentation associated with Z-band disruption as well as myocardial hypertrophy and disarray with interstitial fibrosis. Myocardial fragmentation associated with Z-band disruption may be related to the progression of cardiac dysfunction.
authors.affiliation: Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. Electronic address: hkawano@nagasaki-u.ac.jp.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Kawano
authors.affiliation: Faculty of Science and Engineering, Waseda University, Tokyo, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Kawamura
authors.affiliation: Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishijima
authors.affiliation: Department of Pathology, Shimabara Prefectural Hospital, Shimabara, Japan.
authors.forename: Tomayoshi
authors.initials: T
authors.lastname: Hayashi
authors.affiliation: Department of Pathology, Nagasaki University Hospital, Nagasaki, Japan.
authors.forename: Kuniko
authors.initials: K
authors.lastname: Abe
authors.affiliation: Nagasaki Diagnostic Pathology Clinic, Nagasaki, Japan.
authors.forename: Kioko
authors.initials: K
authors.lastname: Kawai
authors.affiliation: Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Maemura
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D009206:Myocardium / Q000473:pathology*; D009634:Noonan Syndrome / Q000150:complications*; D012518:Sarcomeres / Q000473:pathology*
keywords: Dilated phase hypertrophic cardiomyopathy; Heart failure; Sarcomere