Bini R M, Cleveland D C, Ceballos R, Bargeron L M, Pacifico A D, Kirklin J W
Am J Cardiol. 1984 Aug 1;54(3):369-75. doi: 10.1016/0002-9149(84)90199-1.
Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.
先天性肺静脉狭窄是一种罕见且严重的先天性心脏病。1969年至1982年间，对10例患有此病变的患者进行了研究。2例患者在尸检时被诊断出患有此病；这些患者在先天性心脏病被怀疑之前就已死亡。在8例生前被诊断出患有此病的患者中，6例在临床上被怀疑，2例在心脏导管检查时意外发现。所有患者均接受了手术，5例存活出院。在所有存活者中，接下来的几个月里肺静脉迅速出现进行性再狭窄。5例中有3例接受了再次手术，但进行性再狭窄复发，最终均死于该病。因此，尽管手术部分缓解了肺静脉狭窄，但该病变显然是一种进展性疾病。对于这种致命性病变，没有一种手术修复能够成功治愈或实现长期缓解。
