矫正型大动脉转位合并解剖学左心室退化的外科治疗中期结果

Midterm outcome of surgical therapy for congenitally corrected transposition of the great arteries with morphologically involuted left ventricle

目的 分析单纯肺动脉环缩术与肺动脉环缩术后行双调转术治疗合并解剖学左心室退化矫正型大动脉转位的中期结果.方法 连续入选2005年1月至2012年6月合并解剖学左心室退化的矫正型大动脉转位患儿40例进行回顾性分析.其中,15例患儿在一期肺动脉环缩术后行二期双调转术（解剖根治组）,25例患儿只行肺动脉环缩术（肺动脉环缩组）.行肺动脉环缩术时,患儿年龄中位数为3.1岁,体质量中位数为11.5 kg.术后对两组患儿进行随访.结果 随访（3.4±0.7）年.随访期间,解剖根治组病死率高于肺动脉环缩组[33.3％ （5/15）比4.0％（1/25）,P＜0.05];纽约心脏协会（NYHA）心功能分级为Ⅰ或Ⅱ级的比例[80.0％ （8/10）比95.8％ （24/24）,P＜0.05]和左心室射血分数[（51.4±9.6）％比（61.0±6.4）％,P＜0.01]解剖根治组均小于肺动脉环缩组；肺动脉环缩组无环缩带过紧表现,环缩处压差为（46.9 ±21.5）mmHg（1 mmHg =0.133 kPa）；解剖根治组中3例患儿出现新的中至重度主动脉瓣反流,2例患儿出现严重心律失常.多因素Cox回归分析显示,一期肺动脉环缩术时年龄增大是二期双调转术后死亡（HR=13.30,95％ CI：6.28 ～ 14.02,P＜0.05）和心力衰竭（HR =4.77,95％ CI：2.71 ～8.68,P＜0.05）的危险因素.结论 对于合并解剖学左心室退化的矫正型大动脉转位患儿,单纯肺动脉环缩术的病死率和心功能可能优于肺动脉环缩术后行双调转术.

Objective We followed up all the patients underwent pulmonary artery banding（PAB） with or without the second stage double switch （DS） operation to compare the midterm outcomes between the DS and the prolonged palliative PAB. Methods From January 2005 to June 2012, 40 consecutive patients diagnosed with congenital corrected transposition of the great arteries （cc-TGA） with morphologically involuted left ventricle were included in this retrospective cohort study. Every patient underwent the first stage PAB. The second stage DS was performed in 15 patients （ DS group） and the palliative PAB was prolonged in the other 25 patients （PAB group）. The median age at PAB was 3.1 years （range, 3 months to 8 years）, weight was 11.5 kg （range, 5.0 kg to 21.5 kg）.The patients were followed up after surgery. Results Follow-up time was （ 3.4 ± 0. 7 ） years. Overall mortality was 33.3% （ 5/15 ） in DS group and 4. 0% （1/25） in PAB group （P 〈0. 05）. The ratio of NYHA I - II was 80. 0% （8/10） in DS group and 95. 9% （ 23/24 ） in PAB group （ P 〈 0. 05 ） and the mean left ventricle ejection fraction was （ 51.4 ± 9. 6 ） % in DS group and （ 61.0 ± 6. 4 ） % in PAB group （ P 〈 0. 01 ） during follow up. There was no PAB related cyanosis or ventricular dysfunction and the trans-banding pressure gradient was （46. 9 ± 21.5 ） mmHg（ 1 mmHg = 0. 133 kPa） in PAB group. Echocardiography examination revealed moderate to large aortic regurgitation in 3 patients and severe post-double switch operative arrhythmias was found in 2 patients in DS group. The age at PAB （HR = 13.30,95% CI：6. 28 - 14. 02 ,P 〈0. 05 ） and left ventricle dysfunction （HR = 4. 77, 95% CI：2. 71 -8.68, P 〈 0. 05 ） were identified as the risk factors for mortality after DS. Conclusion Compared to the DS, the prolonged palliative PAB procedure is associated with lower mortality and better cardiac function post operation.