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房间隔缺损的病理生理学与自然病史

Pathophysiology and natural history of atrial septal defect.

作者信息

Le Gloan Laurianne, Legendre Antoine, Iserin Laurence, Ladouceur Magalie

机构信息

Department of Cardiology, Adult Congenital Heart Disease. Institut du thorax, CHU de Nantes, Université de Nantes, boulevard Jacques Monod, Saint-Herblain, France.

Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Department of Cardiology, Adult Congenital Heart Disease Unit, Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C, Paris, France.

出版信息

J Thorac Dis. 2018 Sep;10(Suppl 24):S2854-S2863. doi: 10.21037/jtd.2018.02.80.

DOI:10.21037/jtd.2018.02.80
PMID:30305945
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6174151/
Abstract

Atrial septal defects are among the third most common types of congenital heart disease. This group of malformations includes several types of atrial communications allowing shunting of blood between the systemic and the pulmonary circulations. The nature of shunting across the defect in patients with atrial septal defect is of particular interest. Understanding the underlying mechanisms of interatrial shunts and their natural history will help selecting the best timing for closure, before irreversible cardiac and pulmonary injury occur. This review describes the different pathophysiologic mechanisms involved in the direction and magnitude of blood flow through atrial septal defects. The natural history of an individual born with an isolated atrial septal defect is then discussed, including the impact of a longstanding shunt on survival.

摘要

房间隔缺损是第三类最常见的先天性心脏病。这组畸形包括几种类型的心房交通,允许血液在体循环和肺循环之间分流。房间隔缺损患者经缺损分流的性质尤其令人关注。了解心房分流的潜在机制及其自然病程将有助于在不可逆的心脏和肺部损伤发生之前选择最佳的封堵时机。本综述描述了房间隔缺损血流方向和大小所涉及的不同病理生理机制。随后讨论了孤立性房间隔缺损患者的自然病程,包括长期分流对生存的影响。

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Prevalence and spectrum of NKX2.5 mutations in patients with congenital atrial septal defect and atrioventricular block.先天性房间隔缺损和房室传导阻滞患者中NKX2.5突变的患病率及谱系
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