Cheng C F, Wang J K, Wu M H
Department of Paediatrics, Buddhist Tzu-chi General Hospital, Hualien, Taiwan.
Acta Cardiol. 1999 Oct;54(5):287-90.
We report a case of individual pulmonary vein atresia associated with multiple levels of left heart obstruction, including aortic coarctation, valvular aortic stenosis, and parachute mitral valves with stenosis. The diagnosis of pulmonary vein obstruction is likely to be missed in patients who also have other left heart obstructive diseases, since the latter usually dominates the clinical presentation. We diagnosed the existence of individual pulmonary vein atresia preoperatively via cardiac catheterization. The pulmonary artery angiograms revealed back and forth motion of the dye with no visualization of either a capillary or venous phase on the lesion side. The pulmonary capillary wedge pressure was unevenly elevated and highest on the lesion side. The results were later confirmed by operation and autopsy. Thus, selective pulmonary artery catheterization and angiography remains a good diagnostic tool to rule out the existence of pulmonary vein obstruction in cases which have multiple levels of left heart obstruction.
我们报告一例孤立性肺静脉闭锁合并多级左心梗阻的病例，包括主动脉缩窄、主动脉瓣狭窄以及降落伞样二尖瓣伴狭窄。对于同时患有其他左心梗阻性疾病的患者，肺静脉梗阻的诊断很可能会被漏诊，因为后者通常主导临床表现。我们通过心导管检查在术前诊断出孤立性肺静脉闭锁的存在。肺动脉造影显示造影剂来回运动，病变侧未显示毛细血管期或静脉期。肺毛细血管楔压不均匀升高，病变侧最高。结果随后经手术和尸检证实。因此，对于存在多级左心梗阻的病例，选择性肺动脉导管插入术和血管造影仍是排除肺静脉梗阻存在的良好诊断工具。
