Relief of left ventricular outflow obstruction by cibenzoline in a patient with Fabry's disease--a case report.
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.
Morimoto
Shin-ichiro
S
Division of Cardiology, Department of Internal Medicine, Fujita Health University School of Medicine, Toyoake, Japan. morimoto@fujita-hu.ac.jp
Sugiura
Atsushi
A
Iwase
Masatsugu
M
Kubo
Natsuko
N
Hiramitsu
Shinya
S
Uemura
Akihisa
A
Ohtsuki
Masatsugu
M
Kato
Shigeru
S
Kato
Yasuchika
Y
Hishida
Hitoshi
H
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*
D000889:Anti-Arrhythmia Agents; D007093:Imidazoles; C032151:cifenline