title: Relief of left ventricular outflow obstruction by cibenzoline in a patient with Fabry's disease--a case report.
abstract: A 46-year-old man was admitted for further evaluation of exertional chest discomfort. One family member had experienced sudden death, and 2 others had died of heart failure, including 1 known to have had Fabry's disease. The patient was also diagnosed with Fabry's disease, based on reduced leukocyte alpha-galactosidase A activity, 2.0 nmol/mg protein/hour, as well as endomyocardial biopsy findings of marked sarcoplasmic vacuolization of cardiac muscle cells by light microscopy and lamellated "zebra bodies'' in the cytoplasm shown by electron microscopy. Echocardiography disclosed marked left ventricular hypertrophy and systolic anterior motion of the mitral leaflets. On cardiac catheterization, a left ventricular peak systolic outflow gradient of 50 mm Hg was noted; this decreased to 10 mm Hg following intravenous administration of 100 mg of cibenzoline. It is imperative to recognize the existence of cases with Fabry's disease associated with left ventricular outflow obstruction.
authors.affiliation: Division of Cardiology, Department of Internal Medicine, Fujita Health University School of Medicine, Toyoake, Japan. morimoto@fujita-hu.ac.jp
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Morimoto
authors.forename: Atsushi
authors.initials: A
authors.lastname: Sugiura
authors.forename: Masatsugu
authors.initials: M
authors.lastname: Iwase
authors.forename: Natsuko
authors.initials: N
authors.lastname: Kubo
authors.forename: Shinya
authors.initials: S
authors.lastname: Hiramitsu
authors.forename: Akihisa
authors.initials: A
authors.lastname: Uemura
authors.forename: Masatsugu
authors.initials: M
authors.lastname: Ohtsuki
authors.forename: Shigeru
authors.initials: S
authors.lastname: Kato
authors.forename: Yasuchika
authors.initials: Y
authors.lastname: Kato
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Hishida
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D001706:Biopsy; D004452:Echocardiography; D000795:Fabry Disease / Q000150:complications* / Q000473:pathology; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D014694:Ventricular Outflow Obstruction / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*
chemical_list: D000889:Anti-Arrhythmia Agents; D007093:Imidazoles; C032151:cifenline