Fifteen-Year Follow-up of a Heterozygous Fabry's Disease Patient Associated with Pre-excitation Syndrome

Rie MURATA; Hisato TAKATSU; Toshiyuki NODA; Kazuhiko NISHIGAKI; Kunihiko TSUCHIYA; Genzou TAKEMURA; Motoo KANOH; Akihisa KUNISHIMA; Keiji SANO; Shinya MINATOGUCHI; Hajime TAKAGI; Hisayoshi FUJIWARA

doi:10.2169/internalmedicine.38.476

Rie MURATA, Hisato TAKATSU, Toshiyuki NODA, Kazuhiko NISHIGAKI, Kunihiko TSUCHIYA, Genzou TAKEMURA, Motoo KANOH, Akihisa KUNISHIMA, Keiji SANO, Shinya MINATOGUCHI, Hajime TAKAGI, Hisayoshi FUJIWARA

Author information

Rie MURATA
The Second Department of Internal Medicine, Gifu University School of Medicine
Hisato TAKATSU
The Second Department of Internal Medicine, Gifu University School of Medicine
Toshiyuki NODA
The Second Department of Internal Medicine, Gifu University School of Medicine
Kazuhiko NISHIGAKI
The Second Department of Internal Medicine, Gifu University School of Medicine
Kunihiko TSUCHIYA
The Second Department of Internal Medicine, Gifu University School of Medicine
Genzou TAKEMURA
The Second Department of Internal Medicine, Gifu University School of Medicine
Motoo KANOH
The Second Department of Internal Medicine, Gifu University School of Medicine
Akihisa KUNISHIMA
The Second Department of Internal Medicine, Gifu University School of Medicine
Keiji SANO
The Second Department of Internal Medicine, Gifu University School of Medicine
Shinya MINATOGUCHI
The Second Department of Internal Medicine, Gifu University School of Medicine
Hajime TAKAGI
Dermatology, Gifu University School of Medicine
Hisayoshi FUJIWARA
The Second Department of Internal Medicine, Gifu University School of Medicine

Keywords: hypertrophy, progression, regression, Mahaim fiber

JOURNAL FREE ACCESS

1999 Volume 38 Issue 6 Pages 476-481

DOI https://doi.org/10.2169/internalmedicine.38.476

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Abstract

A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma α-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.
(Internal Medicine 38: 476-481, 1999)

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