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[巨大肺动脉瘤。病例报告及治疗算法建议]

[Giant pulmonary artery aneurysms. Case report and proposal of management algorithm].

作者信息

Radermecker A, Stiennon L, Leroux A, Sooknunden M, Duysinx B, Guiot J, Davin L, Sakalihasan N, Radermecker M A, Defraigne J O

机构信息

Service de Chirurgie cardio-vasculaire et thoracique, CHU Liège, Belgique.

Service de Chirurgie, UCL Mont-Godinne, Belgique.

出版信息

Rev Med Liege. 2019 Feb;74(2):90-94.

Abstract

Pulmonary artery aneurysm is a rare and multiform pathology related to multiple etiologies and therefore different pathophysiological mechanisms. Delineating homogenous sub-groups is a pre-requisite to refine medico-surgical management. The case of a giant PAA without pulmonary hypertension but associated to a dysplastic pulmonary valve is reported. This association could be in some instances the result of a congenital anomaly in the development of both the pulmonary valve and the root creating the conditions for further development of a pulmonary artery aneurysm. Whilst minor forms are usually asymptomatic, they can lead to lethal complications in huge sizes and are frequently associated via pulmonary valve insufficiency to right ventricular dysfunction. This specific association is discussed and a diagnostic algorithm for nosologic classification and management is proposed.

摘要

肺动脉瘤是一种罕见且形态多样的病变,与多种病因相关,因而涉及不同的病理生理机制。划分同质亚组是优化药物和手术治疗的先决条件。本文报道了一例无肺动脉高压但合并发育异常肺动脉瓣的巨大肺动脉瘤病例。这种关联在某些情况下可能是肺动脉瓣和肺动脉根部发育先天性异常的结果,为肺动脉瘤的进一步发展创造了条件。虽然较小的肺动脉瘤通常无症状,但巨大的肺动脉瘤可导致致命并发症,且常因肺动脉瓣关闭不全而并发右心室功能障碍。本文对这种特殊关联进行了讨论,并提出了一种用于疾病分类和管理的诊断算法。

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