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法布里病心肌病合并冠状动脉痉挛的多模态成像

Multimodality Imaging of Fabry Disease Cardiomyopathy Complicated With Coronary Vasospasm.

作者信息

Tahara Tomohiro, Usui Eisuke, Hada Masahiro, Ueno Hiroki, Kakuta Tsunekazu

机构信息

Department of Cardiovascular Medicine, Tsuchiura Kyodo General Hospital, Tokyo, Japan.

出版信息

JACC Case Rep. 2024 Feb 21;29(7):102257. doi: 10.1016/j.jaccas.2024.102257. eCollection 2024 Apr 3.

DOI:10.1016/j.jaccas.2024.102257
PMID:38645294
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11031653/
Abstract

A 41-year-old man with resting angina was diagnosed with a coronary vasospasm and subsequently with Fabry disease exhibiting low serum α-galactosidase A activity. High computed tomography (CT)-derived extracellular volume was detected in the apical inferior wall of the left ventricle suggesting myocardial fibrosis, potentially from vasospasm-related ischemia and/or microvascular dysfunction.

摘要

一名患有静息性心绞痛的41岁男性被诊断为冠状动脉痉挛，随后被诊断为法布里病，其血清α-半乳糖苷酶A活性较低。计算机断层扫描（CT）显示左心室心尖下壁细胞外容积较高，提示心肌纤维化，可能源于血管痉挛相关的缺血和/或微血管功能障碍。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa3/11031653/546b67280b1b/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa3/11031653/3e66b9bb81c3/ga1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa3/11031653/546b67280b1b/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa3/11031653/3e66b9bb81c3/ga1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa3/11031653/546b67280b1b/gr1.jpg

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本文引用的文献

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Cardiac Involvement in Fabry Disease: JACC Review Topic of the Week.《法布瑞病的心脏累及：美国心脏病学会杂志每周评论专题》。
J Am Coll Cardiol. 2021 Feb 23;77(7):922-936. doi: 10.1016/j.jacc.2020.12.024.
2
Unexpectedly High Prevalence of Coronary Spastic Angina in Patients With Anderson-Fabry Disease.意料之外的高患病率：安德森-法布里病患者中的冠状动脉痉挛性心绞痛。
Circ J. 2019 Jan 25;83(2):481-484. doi: 10.1253/circj.CJ-18-0734. Epub 2018 Dec 18.
3
Cardiac T1 Mapping and Extracellular Volume (ECV) in clinical practice: a comprehensive review.
临床实践中的心脏T1映射与细胞外容积（ECV）：全面综述
J Cardiovasc Magn Reson. 2016 Nov 30;18(1):89. doi: 10.1186/s12968-016-0308-4.