Geggel R L, Fried R, Tuuri D T, Fyler D C, Reid L M
J Am Coll Cardiol. 1984 Jan;3(1):193-9. doi: 10.1016/s0735-1097(84)80447-7.
A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and veins in all lobes with or without pulmonary vein stenosis. Arterial changes-muscle extension, medial hypertrophy and decreased arterial size--analyzed quantitatively were found to be similar in all lobes. Venous medial hypertrophy was more marked in obstructed lobes. These anatomic changes are presumably due to fixed venous obstruction in the pulmonary lobes drained by stenotic veins and to high flow in the left upper lobe.
本文描述了一名13个月大的男婴，除左上肺静脉外，所有肺外静脉均发生狭窄，最终死亡。肺动脉楔压正常，这是该病症首次有此报告。尸检发现，无论有无肺静脉狭窄，所有肺叶的肺动脉和肺静脉均有结构变化。定量分析发现，各肺叶的动脉变化——肌层延伸、中层肥厚和动脉管径减小——相似。静脉中层肥厚在梗阻肺叶更为明显。这些解剖学变化可能是由于狭窄静脉引流的肺叶存在固定的静脉梗阻，以及左上肺叶血流过高所致。
