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埃布斯坦畸形

Ebstein Anomaly.

作者信息

Sherwin Elizabeth D, Abrams Dominic J

机构信息

Division of Cardiology, Children's National Health System, 111 Michigan Avenue NW, Washington, DC 20010, USA.

Division of Cardiac Electrophysiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

出版信息

Card Electrophysiol Clin. 2017 Jun;9(2):245-254. doi: 10.1016/j.ccep.2017.02.007.

Abstract

Ebstein anomaly is a rare form of congenital heart disease with a uniquely high prevalence of arrhythmias. The most prevalent arrhythmia mechanisms are intrinsic to the underlying embryologic defects and may manifest at any stage. Current electrophysiological and surgical strategies are well equipped to address these arrhythmia mechanisms, yet despite available technology and a robust understanding of the mechanisms, these cases remain challenging. Surgical techniques that render arrhythmia substrates unreachable mandate comprehensive presurgical electrophysiological assessment and potential ablation. As the population ages, the need to address atrial fibrillation management and risk stratification for sudden cardiac death becomes ever more pertinent.

摘要

埃布斯坦畸形是一种罕见的先天性心脏病,心律失常的发生率特别高。最常见的心律失常机制与潜在的胚胎学缺陷内在相关,可能在任何阶段出现。目前的电生理和外科手术策略能够很好地应对这些心律失常机制,然而,尽管有可用的技术并且对这些机制有深入的了解,但这些病例仍然具有挑战性。使心律失常基质无法触及的手术技术要求进行全面的术前电生理评估和可能的消融。随着人口老龄化,应对心房颤动管理和心脏性猝死风险分层的需求变得越来越迫切。

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