title: Biventricular hypertrophic cardiomyopathy with right ventricular outflow tract obstruction associated with Noonan syndrome in an adult.
abstract: This report describes an adult patient with Noonan syndrome accompanied by biventricular hypertrophic cardiomyopathy causing isolated right ventricular outflow tract obstruction. Biventricular hypertrophic cardiomyopathy causing right- and/or left-side outflow tract obstruction, as well as valvular pulmonary stenosis, is relatively common in infants with Noonan syndrome. However, this condition without a dysplastic pulmonary valve, or indeed any polyvalvular dysplasia, is rare in adults with Noonan syndrome. Treatment with a beta-adrenergic receptor blocking agent improved the patient's symptoms. Because neither the etiologic and prognostic relationship nor the genetic linkage between hypertrophic cardiomyopathy associated with Noonan syndrome and non-syndromic hypertrophic cardiomyopathy is clearly defined, clinicopathological findings and further follow-up may provide important evidence for the pathogenesis of hypertrophic cardiomyopathy.
authors.affiliation: First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan. shin@hsp.md.shinshu-u.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Hayashi
authors.forename: K
authors.initials: K
authors.lastname: Tojyo
authors.forename: S
authors.initials: S
authors.lastname: Uchikawa
authors.forename: T
authors.initials: T
authors.lastname: Momose
authors.forename: T
authors.initials: T
authors.lastname: Misawa
authors.forename: Y
authors.initials: Y
authors.lastname: Yazaki
authors.forename: O
authors.initials: O
authors.lastname: Kinoshita
authors.forename: M
authors.initials: M
authors.lastname: Hongo
authors.forename: K
authors.initials: K
authors.lastname: Kubo
authors.forename: H
authors.initials: H
authors.lastname: Imamura
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D009634:Noonan Syndrome; D014694:Ventricular Outflow Obstruction / Q000150:complications* / Q000503:physiopathology