小儿马凡氏人群中二尖瓣病理与心室异位的关系。

IF 1.4 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Saneeha Shahid, Peter F Aziz, Iqbal El Assaad, Brendan J Burke, Kenneth Zahka, Tara Karamlou, Justin T Tretter, Maryann Patterson, Akash Patel
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引用次数: 0

摘要

成人研究建立了二尖瓣病理,即二尖瓣环分离(MAD)和二尖瓣脱垂(MVP)与室性心律失常之间的联系。儿童马凡氏人群的数据有限。评估(1)MAD与室性异位(VE)、非持续性室性心动过速(NSVT)和室性心动过速(VT)之间的关系;(2)小儿马凡氏人群的MVP和VE, NSVT和VT, (3) MAD和MVP。我们于2001年1月至2022年1月进行了一项回顾性单中心研究,纳入了所有年龄≤21岁且具有心律监测仪的马凡综合征患者。在纳入的32例患者中,12例(38%)为女性,21例(66%)为纤维蛋白1变异阳性。超声心动图的平均年龄为13.5±4.5岁,心脏监测的中位持续时间为58(32.5-190.5)小时。16例(50%)有复合VE(双联体、三胞胎和/或非svt)。14例(44%)有双联体,每个监测者的中位发作数为2(1-4),1例(3%)为多态,6例(19%)具有快速RR (R-R间期)
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Association between Mitral Valve Pathology and Ventricular Ectopy in the Pediatric Marfan Population.

Adult studies establish an association between mitral valve pathology, namely mitral annular disjunction (MAD) and mitral valve prolapse (MVP), and ventricular arrhythmias. Data in the pediatric Marfan population is limited. To assess the association between (1) MAD and ventricular ectopy (VE), non-sustained ventricular tachycardia (NSVT) and ventricular tachycardia (VT); (2) MVP and VE, NSVT and VT and (3) MAD and MVP in the pediatric Marfan population. We carried out a retrospective single center study from January 2001 to January 2022 including all patients with Marfan syndrome who were ≤ 21 years of age and had a cardiac rhythm monitor. Of the 32 patients included, 12 (38%) were female and 21 (66%) had a positive Fibrillin 1 variant. The mean age at echocardiogram was 13.5 ± 4.5 years and median duration of cardiac monitoring was 58 (32.5-190.5) hours. Sixteen (50%) had complex VE (couplets, triplets, and/or NSVT). Fourteen (44%) had couplets with median episodes per monitor of 2 (1-4), 1 (3%) being polymorphic and 6 (19%) with fast RR (R-R interval < 350 ms). Six (19%) had triplets with median episodes per monitor of 1 (1-1) and fast RR in 4 (13%). Four (13%) had NSVT. There is a high prevalence of complex VE in the pediatric Marfan population. MAD and MVP were not associated with complex VE however, all patients with triplets and NSVT had MVP, mostly bileaflet. MAD is positively associated with bileaflet MVP and bileaflet MVP is associated with more ventricular ectopy.

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来源期刊
Pediatric Cardiology
Pediatric Cardiology 医学-小儿科
CiteScore
3.30
自引率
6.20%
发文量
258
审稿时长
12 months
期刊介绍: The editor of Pediatric Cardiology welcomes original manuscripts concerning all aspects of heart disease in infants, children, and adolescents, including embryology and anatomy, physiology and pharmacology, biochemistry, pathology, genetics, radiology, clinical aspects, investigative cardiology, electrophysiology and echocardiography, and cardiac surgery. Articles which may include original articles, review articles, letters to the editor etc., must be written in English and must be submitted solely to Pediatric Cardiology.
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