Endocardial Fibroelastosis Resection: When it Works and When it Does Not

Q2 Medicine Pediatric Cardiac Surgery Annual Pub Date : 2024-01-01 DOI:10.1053/j.pcsu.2023.12.006

Gregor Gierlinger MD, Sitaram M. Emani MD

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Abstract

Endocardial fibroelastosis (EFE) is a thickening of the endocardial layer by accumulation of collagen and elastic fibers. Endothelial to mesenchymal transformation is proposed to be the underlying mechanism of formation. Although EFE can occur in both right and left ventricles, this article will focus on management of left ventricular EFE. Through its fibrous, nonelastic manifestation EFE restricts the myocardium leading to diastolic and systolic ventricular dysfunction and prevents ventricular growth in neonates and infants. The presence of EFE may be a marker for underlying myocardial fibrosis as well. The extent of EFE within the left ventricular cavity can be variable ranging from patchy to confluent distribution. Similarly the depth of penetration and degree of infiltration into myocardium can be variable. The management of EFE is controversial, although resection of EFE has been reported as part of the staged ventricular recruitment therapy. Following resection, EFE recurs and infiltrates the myocardium after primary resection. Herein we review the current experience with EFE resection.

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心内膜纤维瘤切除术--何时有效，何时无效

心内膜纤维细胞增生症（EFE）是心内膜层因胶原蛋白和弹性纤维堆积而增厚。内皮细胞向间充质转化被认为是其形成的基本机制。虽然 EFE 可发生在右心室和左心室，但本文将重点讨论左心室 EFE 的治疗。EFE 以其纤维状、无弹性的表现限制了心肌，导致心室舒张和收缩功能障碍，并阻碍新生儿和婴儿心室的生长。EFE 的存在也可能是潜在心肌纤维化的标志。EFE 在左心室腔内的范围可从斑块状分布到汇合状分布不等。同样，EFE 渗入心肌的深度和程度也不尽相同。尽管有报道称 EFE 的切除是分期心室募集疗法的一部分，但对 EFE 的处理仍存在争议。切除术后，EFE 会复发并浸润心肌。在此，我们回顾了目前 EFE 切除术的经验。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Cardiac Surgery Annual Medicine-Surgery

CiteScore

3.80

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0.00%

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期刊介绍： The Pediatric Cardiac Surgery Annual is a companion to Seminars in Thoracic and Cardiovascular Surgery . Together with the Seminars, the Annual provides complete coverage of the specialty by focusing on important developments in pediatric cardiac surgery. Each annual volume has an expert guest editor who invites prominent surgeons to review the areas of greatest change in pediatric cardiac surgery during the year. Topics include 1) Complete Atrioventricular Canal; 2) New Concepts of Cardiac Anatomy and Function -- The Helical Heart; 3) Valve Reconstruction (Replacement) in Congenital Heart Disease; 4) Evolving Developments in Congenital Heart Surgery.

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Table of Contents Masthead Surgical Heritage: You Had to Be There, Ross: The Comeback Kid Endocardial Fibroelastosis Resection: When it Works and When it Does Not Long-Term Outcomes of Mechanical Aortic Valve Replacement in Children