Angina in fabry disease reflects coronary small vessel disease.

Cristina Chimenti, Emanuela Morgante, Gaetano Tanzilli, Enrico Mangieri, Giuseppe Critelli, Carlo Gaudio, Matteo A. Russo, Andrea Frustaci

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Chest pain is frequently reported in Fabry disease (FD). However, its mechanism and clinical relevance are unclear. METHODS AND RESULTS: Basal troponin I level, exercise stress test, single-photon emission computed tomography imaging with (99m)Tc sestamibi, coronary angiography with thrombolysis in myocardial infarction (TIMI) frame count and left ventricular angiography and endomyocardial biopsy were obtained in 13 patients with FD with angina. Ratio of external to lumen diameter of intramural arteries (E/L ratio), myocyte diameter, and extent of fibrosis were morphometrically evaluated by using tissue sections. Controls for coronary angiography and histology were 25 patients with FD without angina and 20 mitral stenosis patients with normal left ventricular function. Troponin I level was elevated in 6 of the 13 patients. Exercise stress test showed evidence of myocardial ischemia, and single-photon emission computed tomography was positive for stress-induced perfusion defects in all patients with FD with angina. Epicardial coronaries were structurally normal but showed slow flow in all and were associated with aneurisms of posterior left ventricular wall in 3 cases. Histology showed remarkable lumen narrowing of most intramural arteries (mean E/L ratio=3.5+/-1.2; P

Original languageEnglish
Pages (from-to)161-169
Number of pages9
JournalCirculation: Heart Failure
Volume1
Issue number3
DOIs
Publication statusPublished - Sep 2008

ASJC Scopus subject areas

  • Medicine(all)

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