Anatomic variability of tricuspid leaflets and right ventricle in Ebstein's anomaly: Anatomic and morphogenetic considerations

IF 2.3 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
C. Huet, J. Karila-Cohen, B. Bessières, D. Bonnet, L. Houyel
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引用次数: 0

Abstract

Introduction

Ebstein's anomaly of the tricuspid valve (TV) is a congenital cardiac malformation of the tricuspid valve (TV) and right ventricle (RV) characterized by downward displacement of the functional annulus usually involving septal and inferior leaflets. Ebstein's anatomical spectrum is highly variable. This anatomical study aimed to unravel the morphogenetic mechanisms underlying this variability.

Methods

Forty-two hearts exhibiting Ebstein anomaly from the Reference Center for Complex Congenital Heart Defects (CHD) collection were macroscopically analysed: 28 isolated and 14 associated with another CHD. Tricuspid leaflets, right ventricle (RV), and associated lesions were analysed. Measurements were related to fetal heart reference values according to gestational age. Specimens were classified depending on the distal insertion of the anterior leaflet: linear, hyphenated or focal.

Results

Among isolated Ebstein hearts, distal insertion of the anterior leaflet was linear in 36%, hyphenated in 29% and focal in 36%. Linear insertion was always associated with anterior TV orifice and absent inferior leaflet, with absent septal leaflet in 80%. Atrialisation (thin, paper-like, inferior RV wall) was found in 90% of the linear forms and 37.5% of the hyphenated forms, which were associated with various degrees of inferior and septal leaflets non-delaminationanomalies. In focal forms, only the septal leaflet was involved, and there was no atrialisation. Ventricular septal defect was found in 3 specimens, Uhl-like RV anterior wall in 2, including 1 with left ventricle non compaction. Associated Ebstein anomalies were predominantly found in pulmonary atresia with intact ventricular septum; there was no atrialisation and septal leaflet only was involved.

Conclusion

Ebstein anomaly corresponds to an arrest in TV development, early in linear forms, late in focal forms, intermediate in hyphenated forms. Atrialisation was always associated with absent inferior leaflet, underlining developmental interaction between endocardial cushions and myocardium at the RV inferior wall.

爱布斯坦氏畸形患者三尖瓣叶和右心室的解剖变异:解剖和形态发生学方面的考虑因素
导言三尖瓣(TV)埃布斯坦氏畸形是三尖瓣(TV)和右心室(RV)的先天性心脏畸形,其特征是功能瓣环向下移位,通常涉及隔叶和下叶。埃布斯坦氏畸形的解剖谱差异很大。方法对复杂先天性心脏缺损参考中心(CHD)收集的 42 颗表现为 Ebstein 异常的心脏进行了宏观分析:28 颗单独存在,14 颗与其他 CHD 伴发。对三尖瓣叶、右心室(RV)和相关病变进行了分析。根据胎龄,测量值与胎儿心脏参考值相关。根据前叶远端插入情况对标本进行分类:直线型、连线型或病灶型。结果在孤立的 Ebstein 心脏中,前叶远端插入为直线型的占 36%,连线型的占 29%,病灶型的占 36%。线性插入总是与前TV口和缺失的下叶相关,80%的人缺失室间隔小叶。90%的线性插入型和37.5%的连线插入型患者出现心房化(薄如纸张的下腔室壁),并伴有不同程度的下叶和室间隔小叶无脱垂异常。在局灶型中,只有室间隔小叶受累,且没有心房化。3例标本中发现室间隔缺损,2例发现Uhl样RV前壁,其中1例伴有左心室非压缩。相关的 Ebstein 异常主要见于肺动脉闭锁,室间隔完整;没有心房化,仅室间隔小叶受累。心房化总是与下叶缺失有关,强调了心内膜垫和 RV 下壁心肌在发育过程中的相互作用。
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来源期刊
Archives of Cardiovascular Diseases
Archives of Cardiovascular Diseases 医学-心血管系统
CiteScore
4.40
自引率
6.70%
发文量
87
审稿时长
34 days
期刊介绍: The Journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles and editorials. Topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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