Department of Pediatrics, Sejong General Hospital, Bucheon, Korea.
Korean Circ J. 2010 Jan;40(1):31-5. doi: 10.4070/kcj.2010.40.1.31. Epub 2010 Jan 27.
Recent surgical results from total anomalous pulmonary venous return (TAPVR) are reported to have improved; however, mortality and morbidity are still high in the univentricles. This review was performed in order to demonstrate surgical results from TAPVR for recent years in a single institute.
A total of 53 patients with TAPVR underwent surgery between January 2000 and December 2008. Preoperative anatomy and hemodynamics were evaluated by 2-dimensional echocardiogram. We reviewed medical records on preoperative management, age, and body weight at time of surgery, postoperative mortality, and pulmonary venous obstruction.
The study included 36 biventricle patients and 17 univentricle patients. Median age and weight at time of surgery were 26 days and 3.5 kg, respectively. During the mean follow up period of 48.2 months, a total of 11 deaths and 8 early deaths occurred. Postoperative pulmonary venous stenosis occurred in 8 patients. Among 36 biventricle patients, mortality occurred in 1 patient and postoperative pulmonary venous stenosis occurred in 6 patients. No statistical significances were observed between postoperative pulmonary venous stenosis and other factors. Among 17 univentricule patients, mortality was 10, including 7 early deaths, and postoperative pulmonary venous stenosis occurred in 2 patients. Combined palliations were modified Blalock-Taussig (BT) shunts in 7, pulmonary artery banding (PAB) in 3, bidirectional cavopulmonary shunt in 5, and the Fontan procedure in 1. The highest mortality was recorded in patients with a modified BT shunt (85.7%) and next in patients with PAB (66.6%). Mortality was not related to anatomy of TAPVR, nor preoperative obstruction, but with significant age and weight at time of surgery.
Despite improvement in operative results from TAPVR, surgery in univentricles is highly risky, especially with low weight and early age, with concomitant palliation.
全肺静脉异位连接（TAPVR）的近期手术结果有改善，但单心室患者的死亡率和发病率仍较高。本研究旨在展示单中心近年来 TAPVR 的手术结果。
2000 年 1 月至 2008 年 12 月，53 例 TAPVR 患者接受手术治疗。二维超声心动图评估术前解剖和血流动力学。我们回顾了术前管理、手术时年龄和体重、术后死亡率和肺静脉梗阻的病历。
研究包括 36 例双心室患者和 17 例单心室患者。手术时的中位年龄和体重分别为 26 天和 3.5kg。在 48.2 个月的平均随访期间，共有 11 例死亡和 8 例早期死亡。8 例患者术后发生肺静脉狭窄。36 例双心室患者中，1 例死亡，6 例术后肺静脉狭窄。术后肺静脉狭窄与其他因素之间无统计学差异。17 例单心室患者中，10 例死亡，包括 7 例早期死亡，2 例术后肺静脉狭窄。改良 Blalock-Taussig（BT）分流术 7 例，肺动脉环缩术 3 例，双向腔静脉肺动脉吻合术 5 例，Fontan 手术 1 例。改良 BT 分流术患者死亡率最高（85.7%），其次是肺动脉环缩术患者（66.6%）。死亡率与 TAPVR 的解剖结构无关，也与术前梗阻无关，而是与手术时的年龄和体重显著相关。
尽管 TAPVR 的手术结果有所改善，但单心室手术风险仍然很高，尤其是在体重低、年龄小的情况下，需要同时进行姑息治疗。
