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57例完全性肺静脉异位连接尸检病例中的肺血管疾病

Pulmonary vascular disease in 57 necropsy cases of total anomalous pulmonary venous connection.

作者信息

Petersen R C, Edwards W D

出版信息

Histopathology. 1983 Jul;7(4):487-96. doi: 10.1111/j.1365-2559.1983.tb02262.x.

DOI:10.1111/j.1365-2559.1983.tb02262.x
PMID:6884996
Abstract

In a semi-quantitative necropsy study, total anomalous pulmonary venous connection (TAPVC) was an isolated anomaly in 35 cases and was associated with asplenia and either pulmonary stenosis or pulmonary atresia in 22 cases. A comparison of the two groups showed pulmonary venous obstruction (26% vs. 4%), a small interatrial communication (51% vs. 4%), a patent ductus arteriosus or operative shunt (40% vs. 64%), medial hypertrophy of muscular pulmonary arteries (80 vs. 23%), muscularization of arterioles (80% vs. 23%), capillary engorgement (60% vs. 27%) alveolar wall thickening (29% vs. 0%), interlobular septal oedema (34% vs. 14%), dilated lymphatics (80% vs. 45%), arterialization of pulmonary veins (86% vs. 68%), medial hypertrophy of veins (26% vs. 0%), and venous dilatation (74% vs. 50%). Only one patient, a 5-year-old girl with isolated TAPVC, had severe plexogenic pulmonary arteriopathy. In the remainder, the pulmonary lesions were those generally associated with reversible pulmonary venous hypertension; they were most striking in patients with pulmonary venous obstruction, and were least prominent in patients with pulmonary stenosis or pulmonary atresia.

摘要

在一项半定量尸检研究中,完全性肺静脉异位连接(TAPVC)在35例中为孤立性异常,在22例中与无脾以及肺动脉狭窄或肺动脉闭锁相关。两组比较显示,肺静脉梗阻(26% 对4%)、小的房间隔交通(51% 对4%)、动脉导管未闭或手术分流(40% 对64%)、肌性肺动脉中层肥厚(80对23)、小动脉肌化(80% 对23%)、毛细血管充血(60% 对27%)、肺泡壁增厚(29% 对0%)、小叶间隔水肿(34% 对14%)、淋巴管扩张(80% 对45%)、肺静脉动脉化(86% 对68%)、静脉中层肥厚(26% 对0%)以及静脉扩张(74% 对50%)。仅1例患者,一名患有孤立性TAPVC的5岁女孩,有严重的丛状肺血管病。在其余患者中,肺部病变为通常与可逆性肺静脉高压相关的病变;它们在肺静脉梗阻患者中最为显著,而在肺动脉狭窄或肺动脉闭锁患者中最不明显。

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