Morales David L S, Braud Brandi E, Booth Justin H, Graves Daniel E, Heinle Jeffrey S, McKenzie E Dean, Fraser Charles D
Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA.
Ann Thorac Surg. 2006 Nov;82(5):1621-7; discussion 1627-8. doi: 10.1016/j.athoracsur.2006.05.053.
Survival after repair of total anomalous pulmonary venous return in patients with heterotaxy syndrome has consistently been reported in the literature to be far inferior to the results obtained in nonheterotaxy patients. The outcomes of heterotaxy patients at Texas Children's Hospital undergoing repair of total anomalous pulmonary venous return were examined and compared with those of nonheterotaxy patients.
Between July 7, 1995, and November 22, 2005, 122 consecutive patients underwent primary repair of total anomalous pulmonary venous return. Characteristics were as follows: mean age, 0.35 +/- 1.8 years; mean weight, 4.3 +/- 2.8 kg; single ventricle, 40 (33%); heterotaxy, 38 (31%); pulmonary atresia, 13 (11%). Concomitant procedures included pulmonary artery banding in 4 (3%), systemic-to-pulmonary shunt in 14 (12%), bidirectional Glenn in 11 (9%), and other in 16 (14%).
With a mean follow-up of 2.6 +/- 2.5 years including all patients, overall 30-day and 5-year survival was 93% and 86%, respectively; for heterotaxy patients it was 84% and 79%; and for nonheterotaxy patients it was 96% and 89%. Heterotaxy and nonheterotaxy survival curves were not significantly different (p> 0.05). Overall, there were 12 (9.8%) patients who required reoperation for pulmonary vein stenosis, at a mean of 5.1 +/- 5.5 months postoperatively. Seven (58%) reoperations were in heterotaxy patients. Rate of pulmonary vein reoperation is statistically greater (p < 0.05) for heterotaxy patients than nonheterotaxy patients. At last follow-up, all patients were in New York Heart Association class II or less. Using Cox proportional hazards models, pulmonary atresia and need for systemic-to-pulmonary shunt were risk factors for death (not heterotaxy). Single ventricle, operation after July 2000, and need for systemic-to-pulmonary shunt were risk factors for pulmonary vein reoperation.
At midterm follow-up, heterotaxy patients undergoing total anomalous pulmonary venous return repair have a higher pulmonary vein reoperative rate but a comparable overall survival to nonheterotaxy patients.
文献一直报道，内脏异位综合征患者完全性肺静脉异位引流修复术后的生存率远低于非内脏异位患者。我们对在德克萨斯儿童医院接受完全性肺静脉异位引流修复术的内脏异位患者的预后进行了研究，并与非内脏异位患者的预后进行了比较。
1995年7月7日至2005年11月22日期间，122例连续患者接受了完全性肺静脉异位引流一期修复术。患者特征如下：平均年龄0.35±1.8岁；平均体重4.3±2.8千克；单心室40例（33%）；内脏异位38例（31%）；肺动脉闭锁13例（11%）。同期手术包括4例（3%）肺动脉环扎术、14例（12%）体肺分流术、11例（9%）双向格林分流术以及16例（14%）其他手术。
所有患者平均随访2.6±2.5年，总体30天和5年生存率分别为93%和86%；内脏异位患者分别为84%和79%；非内脏异位患者分别为96%和89%。内脏异位和非内脏异位患者的生存曲线无显著差异（p>0.05）。总体而言，有12例（9.8%）患者因肺静脉狭窄需要再次手术，平均在术后5.1±5.5个月。其中7例（58%）再次手术的是内脏异位患者。内脏异位患者肺静脉再次手术率在统计学上高于非内脏异位患者（p<0.05）。在最后一次随访时，所有患者的心功能分级均为纽约心脏协会二级或更低。使用Cox比例风险模型，肺动脉闭锁和体肺分流需求是死亡的危险因素（非内脏异位）。单心室、2000年7月后手术以及体肺分流需求是肺静脉再次手术的危险因素。
在中期随访中，接受完全性肺静脉异位引流修复术的内脏异位患者肺静脉再次手术率较高，但总体生存率与非内脏异位患者相当。
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