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Unexpectedly High Prevalence of Coronary Spastic Angina in Patients With Anderson-Fabry Disease

Yuya Kitani1, Naoki Nakagawa1, Naka Sakamoto1

  • 1Division of Cardiology, Nephrology, Pulmonology and Neurology, Department of Internal Medicine, Asahikawa Medical University.

Circulation Journal : Official Journal of the Japanese Circulation Society
|December 21, 2018
PubMed

Insights

Coronary spastic angina (CSA) is surprisingly common in patients with Anderson-Fabry disease (AFD). This study found an 89% prevalence of CSA in AFD patients, suggesting a significant link between these conditions.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Anderson-Fabry disease (AFD) is a rare genetic disorder.
  • Coronary spastic angina (CSA) has been observed in some AFD patients, but its prevalence is unknown.

Purpose of the Study:

  • To determine the prevalence of coronary spastic angina (CSA) in patients with Anderson-Fabry disease (AFD).

Main Methods:

  • Nine patients from five independent AFD pedigrees underwent acetylcholine-induced provocation testing.
  • Testing followed Japanese guidelines for diagnosing and treating CSA.

Main Results:

  • Coronary spasms were provoked in 8 out of 9 (89%) AFD patients.
  • Provocation was associated with typical symptoms and ECG ischemic changes.

Conclusions:

  • An unexpectedly high prevalence of CSA was found in patients with AFD.
  • This suggests a strong association between Anderson-Fabry disease and coronary spastic angina.
Abstract

Keywords:
Acetylcholine-induced provocation testAnderson-Fabry diseaseCoronary spastic angina

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