Journal
CIRCULATION JOURNAL
Volume 83, Issue 2, Pages 481-+Publisher
JAPANESE CIRCULATION SOC
DOI: 10.1253/circj.CJ-18-0734
Keywords
Acetylcholine-induced provocation test; Anderson-Fabry disease; Coronary spastic angina
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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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