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[先天性肺淋巴管扩张症。1例伴有肺静脉回流完全受阻及显著间质性肺气肿的新病例]

[Congenital pulmonary lymphangiectasis. One new case with complete blockage of the return venous pulmonary circulation and marked interstitial emphysema].

作者信息

Lallemand D, Huault G

出版信息

Nouv Presse Med. 1977 May 21;6(21):1861-3.

PMID:876832
Abstract

In this unusual case of bilateral congenital pulmonary lymphangiectasia with complete block of pulmonary venous return, the radiological diagnosis was made difficult by the association of the lymphangiectasia with bilateral interstitial emphysema. Routine dissection of the heart, considered to be normal since the cardio-thoracic index was only 0.45, relealed the true cause of death. The heart was the site of extreme hypoplasia of the left heart with complete mitral and aortic atresia. The ostium secundum being completely closed, the left atrium, after receiving the pulmonary veins, was completely blocked. It communicated neither with the right atrium, nor with the non-existent left ventricule.

摘要

在这个双侧先天性肺淋巴管扩张症合并肺静脉回流完全受阻的罕见病例中,淋巴管扩张症与双侧间质性肺气肿并存,使得放射学诊断变得困难。心脏的常规解剖显示了真正的死因,尽管心胸指数仅为0.45,心脏当时被认为是正常的。心脏存在严重的左心发育不全,伴有二尖瓣和主动脉完全闭锁。继发孔完全闭合,左心房在接纳肺静脉后完全受阻。它既不与右心房相通,也不与不存在的左心室相通。

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