Relief of left ventricular outflow obstruction by cibenzoline in a patient with Fabry's disease: A case report

Shin Ichiro Morimoto, Atsushi Sugiura, Masatsugu Iwase, Natsuko Kubo, Shinya Hiramitsu, Akihisa Uemura, Masatsugu Ohtsuki, Shigeru Kato, Yasuchika Kato, Hitoshi Hishida

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

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 α-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.

Original languageEnglish
Pages (from-to)241-245
Number of pages5
JournalAngiology
Volume57
Issue number2
DOIs
Publication statusPublished - 03-2006

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

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