Circulation Journal
Online ISSN : 1347-4820
Print ISSN : 1346-9843
ISSN-L : 1346-9843

This article has now been updated. Please use the final version.

Unexpectedly High Prevalence of Coronary Spastic Angina in Patients With Anderson-Fabry Disease
Yuya KitaniNaoki NakagawaNaka SakamotoToshiharu TakeuchiFumihiko TakahashiKen MomosakiKimitoshi NakamuraFumio EndoHiroki MaruyamaNaoyuki Hasebe
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JOURNAL FREE ACCESS FULL-TEXT HTML Advance online publication
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Article ID: CJ-18-0734

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Abstract

Background: Although we and others have reported cases of patients with Anderson-Fabry disease (AFD) complicated by coronary spastic angina (CSA), the prevalence of CSA in these patients remains unknown.

Methods and Results: We performed the acetylcholine-induced provocation test, according to the Japanese guidelines for the diagnosis and treatment of patients with CSA, in 9 consecutive patients having 5 independent AFD pedigrees. Coronary spasms were provoked in conjunction with symptoms and ECG ischemic changes in 8 of 9 (89%) patients with AFD.

Conclusions: We found an unexpectedly high prevalence of CSA in patients with AFD.

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© 2018 THE JAPANESE CIRCULATION SOCIETY
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