Caldarone C A, Najm H K, Kadletz M, Smallhorn J F, Freedom R M, Williams W G, Coles J G
Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto and University of Toronto Faculty of Medicine, Canada.
Ann Thorac Surg. 1998 Nov;66(5):1514-20. doi: 10.1016/s0003-4975(98)00952-7.
Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy.
Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques.
Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years' follow-up. Stenting was uniformly unsuccessful.
In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.
完全性肺静脉异位引流修复术后肺静脉进行性狭窄常常难以通过手术治疗。
对170例连续接受完全性肺静脉异位引流治疗的患者进行回顾性分析，确定了13例修复后肺静脉狭窄患者。分析术前和手术数据以明确狭窄进展模式和手术技术的疗效。
13例患者共进行了17次再次手术。修复后肺静脉狭窄在心上型和混合型中最为常见（p < 0.02）。所有4例单侧狭窄患者均存活，其中2例狭窄进展导致几乎完全性单侧肺静脉闭塞。9例双侧病变患者中有6例死亡（与单侧病变相比，p < 0.05）；3例幸存者中有2例采用了一种新的技术修复，即创建无缝合线的新心房，在1.8年的随访中无再狭窄证据。支架置入均未成功。
在单侧狭窄中，疾病进展可能可通过单肺灌注丧失而存活。尽管双侧病变在大多数情况下是致命的，但创建无缝合线的新心房已显示出短期内无疾病进展。
