48例心房异构合并心脏畸形外科治疗后临床转归

Surgical Outcome for Atrial Isomerism Associated with Cardiac Abnormalities in 48 Pantients

目的本研究旨在总结心房异构患者外科手术处理及临床转归。方法总结首都医科大学附属北京安贞医院小儿心脏中心2010—2012年48例心房异构合并心脏畸形，并行外科手术治疗的患儿，分为左心房异构和右心房异构两组。术后随访1～60个月，分析不同心房异构的疾病特点与外科手术治疗后临床转归。结果48例患儿平均年龄65．54±65．10个月，体重平均16．13±9．00kg，左心房异构9例（18．8％），右心房异构39例（81．2％），2例行双心室治疗，46例行单心室治疗，Glenn术在右心房异构中应用明显多于左心房异构（P：0．045），呼吸机使用时间左心房异构患者长于右心房异构患儿（P=0．029）。全组病例死亡8例，1例左心房异构，7例右心房异构。48例患儿随访5年生存率左心房异构患儿91．67％，右心房异构患儿45．38％。结论心房异构患者往往合并复杂心脏畸形，其中左心房异构少于右心房异构，左心房异构多行双心室治疗，而右心房异构多行单心室治疗，例如Glenn手术应用较多，随访5年生存率左心房异构优于右心房异构。

Objective To review surgical outcome in children with atrial isomerism. Methods Between 2010 and 2012, 48 pa- tients diagnosed with atrial isomerism were treated in our institution. Patients were divided into two groups, group 1 with left atrial isomer- ism, group 2 with right atrial isomerism. The patients were followed up froml to 30 months. Results The average age was 65.54 ± 65.10 month and the average weight was 16.13± 9.00kg. Left atrial isomerism was diagnosed in 9 while right atial isomerism in 39, the manage- ment including single ventricle palliation in 46 and biventricle repair in 2. Bidirectional Glenn procedure was more common in right atrial isomerism than left atrial isomerism （ P = O. 045 ）. Mechanical ventilation time was significantly longer in left atrial isomerism. There were 8 deaths occurred with 1 in left atrial isomerism and 7 in right atrial isomerism. Overall survival in 60 months follow - up was 91.67% in left atrial isomerism and 45.38% in right atrial isomerism. Conclusion Patients with atrial isomerism are often associated with complex cardiac malformations, of which left atrial isomerism less than right atrial isomerism. And it is often treated with biventrieular, but right atrial isomerism is often treated with single ventricle, such as Glenn operation. Left atrial isomerism shows a better outcome in 5 - years follow - up.