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马方综合征青少年患者中二尖瓣环分离与致命性室性心律失常相关

Mitral Annular Disjunction Associated With Fatal Ventricular Arrhythmia in an Adolescent With Marfan Syndrome.

作者信息

Carr Karen, Yetman Anji, Garg Ruchira

机构信息

Department of Pediatrics, University of California-Los Angeles, Los Angeles, California, USA.

Internal Medicine and Pediatric Cardiology, Division of Cardiovascular Medicine, Children's Hospital & Medical Center, University of Nebraska Medical Center, Omaha, Nebraska, USA.

出版信息

JACC Case Rep. 2021 Oct 6;3(13):1551-1556. doi: 10.1016/j.jaccas.2021.08.006.

DOI:10.1016/j.jaccas.2021.08.006
PMID:34693358
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8511475/
Abstract

We discuss an adolescent with Marfan syndrome and a previous aortic valve-sparing root replacement who was found to have mitral annular disjunction on surveillance cardiac imaging in the setting of recurrent palpitations. Ambulatory heart rate monitoring incidentally captured a fatal ventricular arrhythmia, a well-recognized but underappreciated cause of sudden cardiac death in patients with Marfan syndrome. ().

摘要

我们讨论了一名患有马凡综合征且曾接受保留主动脉瓣的根部置换术的青少年,该患者在反复心悸的情况下,经心脏超声监测发现二尖瓣环分离。动态心率监测偶然捕捉到一次致命的室性心律失常,这是马凡综合征患者心脏性猝死的一个公认但未得到充分重视的原因。()

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/d25d1768425b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/d2f538060d75/fx1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/ba54778cabba/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/c5407747342b/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/d25d1768425b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/d2f538060d75/fx1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/ba54778cabba/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/c5407747342b/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e7ac/8511475/d25d1768425b/gr3.jpg

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本文引用的文献

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Myxomatous Mitral Valve Disease with Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical and Functional Significance of the Coincidence.伴有二尖瓣脱垂和二尖瓣环分离的黏液样二尖瓣疾病:两者并存的临床和功能意义
J Cardiovasc Dev Dis. 2021 Jan 24;8(2):9. doi: 10.3390/jcdd8020009.
2
Association of Mitral Annular Disjunction With Premature Cardiac Mortality in a Large Series of Autopsies.大量尸检中二尖瓣环分离与心脏过早死亡的关联
J Am Coll Cardiol. 2021 Jan 5;77(1):102-104. doi: 10.1016/j.jacc.2020.10.051.
3
Multimodality imaging assessment of mitral annular disjunction in mitral valve prolapse.
Eur Heart J Cardiovasc Imaging. 2024 Aug 26;25(9):1306-1314. doi: 10.1093/ehjci/jeae125.
4
Mitral Annular Disjunction: An Under-Recognized Entity in Pediatrics.二尖瓣环分离:儿科中一个未被充分认识的病症。
JACC Case Rep. 2024 Mar 21;29(9):102297. doi: 10.1016/j.jaccas.2024.102297. eCollection 2024 May 1.
二尖瓣脱垂患者二尖瓣环分离的多模态影像学评估。
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4
Features of Marfan syndrome not listed in the Ghent nosology - the dark side of the disease.《根特分类法》未列出的马凡综合征特征——该疾病的阴暗面。
Expert Rev Cardiovasc Ther. 2019 Dec;17(12):883-915. doi: 10.1080/14779072.2019.1704625.
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Mitral annular disjunction: A systematic review of the literature.二尖瓣环分离:文献系统综述
Echocardiography. 2019 Aug;36(8):1549-1558. doi: 10.1111/echo.14437. Epub 2019 Aug 5.
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