Current Surgical Outcomes of Congenital Heart Surgery for Patients With Down Syndrome in Japan
Current Surgical Outcomes of Congenital Heart Surgery for Patients With Down Syndrome in Japan
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DOI：
10.1253/circj.cj-17-0483
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发表时间：
2018-02-01
影响因子：
3.3
通讯作者：
Takamoto, Shinichi
中科院分区：
文献类型：
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作者：
Hoashi, Takaya;Hirahara, Norimichi;Takamoto, Shinichi
背景资料：方法和结果：在日本先天性心血管外科数据库（JCCVSD）2008年至2012年登记的29，087例手术中，有2，651例（9%）是为唐氏综合征患者实施的。其中，5例重大双心室修复术[室间隔缺损修复术（n=752）、房室间隔缺损修复术（n=452）、动脉导管未闭闭合术（n=184）、房间隔缺损修复术（n=167）、法洛四联症（TOF）修复术（n=108）]，同时选择2种主要的单心室姑息治疗方法[双向Glenn术（n=21）和Fontan术（n=25）]并比较其疗效。与非唐氏综合征患者相比，唐氏综合征患者的所有5种主要双心室修复术和双向Glenn的90天和住院死亡率同样较低。另一方面，唐氏综合征患者Fontan手术后的死亡率明显高于非唐氏综合征患者（42/1，558 =2.7% vs. 3/25= 12.0%，P=0.005）。虽然肺动脉高压的强化管理是必不可少的，对JCCVSD的分析显示，唐氏综合征患者接受5次主要双心室手术和双向Glenn后，早期预后良好。Fontan手术对唐氏综合征患者的适应症应慎重决定。
Background: Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results: Of 29,087 operations between 2008 and 2012 registered in the Japan Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures [ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108)], as well as 2 major single ventricular palliations [bidirectional Glenn (n=21) and Fontan operation (n=25)] were selected and their outcomes were compared. The 90-day and in-hospital mortality rates for all 5 major biventricular repair procedures and bidirectional Glenn were similarly low in patients with Down syndrome compared with patients without Down syndrome. On the other hand, mortality after Fontan operation in patients with Down syndrome was significantly higher than in patients without Down syndrome (42/1,558=2.7% vs. 3/25=12.0%, P=0.005).Conclusions: Although intensive management of pulmonary hypertension is essential, analysis of the JCCVSD revealed favorable early prognostic outcomes after 5 major biventricular procedures and bidirectional Glenn in patients with Down syndrome. Indication of the Fontan operation for patients with Down syndrome should be carefully decided.