van Son J A, Danielson G K, Puga F J, Edwards W D, Driscoll D J
Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Ann Thorac Surg. 1995 Jul;60(1):144-50. doi: 10.1016/0003-4975(95)00325-f.
Congenital pulmonary vein stenosis is a rare cause of obstruction of pulmonary venous blood flow with a high mortality. Acquired pulmonary vein stenosis is an equally serious condition.
Eight patients (age range, 3 months to 43 years; median age, 1.5 years) underwent surgical relief of pulmonary vein stenosis. Two had congenital pulmonary vein stenosis, 5 had pulmonary vein stenosis that was acquired after surgical treatment of total anomalous pulmonary venous connection, and 1 had pulmonary vein stenosis associated with idiopathic mediastinal fibrosis and calcification.
One infant died 2 months after correction of acquired pulmonary vein stenosis. At follow-up extending to 16 years (median follow-up, 6.5 years), 6 patients are in New York Heart Association functional class I, and 1 patient is in class II.
In view of the dismal natural history of untreated pulmonary vein stenosis, prompt surgical relief of the stenosis may be a rewarding undertaking.
先天性肺静脉狭窄是肺静脉血流梗阻的一种罕见原因，死亡率很高。获得性肺静脉狭窄也是一种同样严重的病症。
8例患者（年龄范围3个月至43岁；中位年龄1.5岁）接受了肺静脉狭窄的手术治疗。2例为先天性肺静脉狭窄，5例为完全性肺静脉异位连接手术治疗后获得性肺静脉狭窄，1例为与特发性纵隔纤维化和钙化相关的肺静脉狭窄。
1例婴儿在获得性肺静脉狭窄矫正术后2个月死亡。随访至16年（中位随访时间6.5年），6例患者纽约心脏协会心功能分级为I级，1例为II级。
鉴于未经治疗的肺静脉狭窄自然病程不佳，及时进行狭窄的手术解除可能是一项值得的工作。
