与马凡综合征相关的儿童心肌病证据。

IF 5.1 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
Heart Pub Date : 2024-06-17 DOI:10.1136/heartjnl-2024-323922
Justin Weigand, Sara Stephens-Novy, Shagun Sachdeva, Tam T Doan, Abigail Yasso, Shaine A Morris
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引用次数: 0

摘要

目的:马凡综合征(MFS)相关心肌病是指容量负荷无法解释的心室扩张和功能障碍,在儿童中的定义尚不明确。本研究使用心脏磁共振成像(cMRI)评估了小儿 MFS 的心室大小和功能:这项回顾性队列研究检查了 MFS 患者 2.二尖瓣和主动脉瓣反流与左心室搏出量的综合指数:58名患者(60%为男性)接受了评估。初次接受 cMRI 检查时的中位年龄为 13.6 岁(IQR 10.0-15.8 岁)。在初次接受 cMRI 时左心室容积负荷缺失/轻微的患者(44 人,76%)中,左心室舒张末期容积(EDV)指数显著高于正常值(中位数 z 值为 1.8,IQR 为 0.6-3.5,p):心室扩张和EF值降低在MFS患儿中很常见,并且在无/轻度左心室容量负荷的情况下也会发生,这提示存在内在性心肌病。MVP可能与心肌病有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Evidence of cardiomyopathy associated with Marfan syndrome in children.

Objective: Marfan syndrome (MFS)-associated cardiomyopathy, defined as ventricular dilation and dysfunction unexplained by volume loading, is not well defined in children. This study evaluated ventricular size and function in paediatric MFS using cardiac MRI (cMRI).

Methods: This retrospective cohort study examined patients with MFS <19 years old at first cMRI. Left ventricular (LV) ejection fraction (EF) <55% was considered abnormal, as were z-scores >2. Combined mitral and aortic regurgitation indexed to LV stroke volume <20% defined absent/mild volume load. Biventricular volumes and EF on serial cMRI studies were compared with normative paediatric cMRI values, with measures converted to z-scores as appropriate. Longitudinal changes in volumes and EF were evaluated by mixed linear regression. Associations between ventricular, aortic and mitral characteristics were evaluated.

Results: 58 patients (60% male) were evaluated. Median age at initial cMRI was 13.6 years (IQR 10.0-15.8 years). Among patients with absent/mild LV volume load at initial cMRI (n=44, 76%), indexed LV end-diastolic volume (EDV) was significantly increased above normative values (median z-score 1.8, IQR 0.6-3.5, p<0.0001) and LVEF was abnormal in 48% (21/44). In the absence of volume loading, mitral valve prolapse (MVP) was associated with larger ventricular volumes and lower LVEF. Among those with serial cMRIs, LVEF and EDV z-scores did not significantly change over a mean follow-up time between cMRI studies of 1.5 years.

Conclusion: Ventricular dilation and reduced EF are common in children with MFS and occur with no/mild LV volume load, suggesting intrinsic cardiomyopathy. MVP may be associated with cardiomyopathy.

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来源期刊
Heart
Heart 医学-心血管系统
CiteScore
10.30
自引率
5.30%
发文量
320
审稿时长
3-6 weeks
期刊介绍: Heart is an international peer reviewed journal that keeps cardiologists up to date with important research advances in cardiovascular disease. New scientific developments are highlighted in editorials and put in context with concise review articles. There is one free Editor’s Choice article in each issue, with open access options available to authors for all articles. Education in Heart articles provide a comprehensive, continuously updated, cardiology curriculum.
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