Wada Naoki, Takahashi Yukihiro, Ando Makoto, Park In-Sam, Sasaki Takashi
Department of Cardiovascular Surgery, Sakakibara Heart Institute, Japan Promotion Society for Cardiovascular Diseases, 3-16-1 Asahi-cho, Fuchu, Tokyo, 183-0003, Japan.
Gen Thorac Cardiovasc Surg. 2008 Mar;56(3):104-8. doi: 10.1007/s11748-007-0201-z. Epub 2008 Mar 14.
There is a paucity of information regarding appropriate management of children with Down's syndrome and a functional single ventricle. We report the results of bidirectional superior cavopulmonary shunts in six patients with Down's syndrome with a functional single ventricle.
Between January 1991 and December 2004, we identified six patients with Down's syndrome among 263 who had undergone bidirectional superior cavopulmonary shunts (BCPSs). There were four males and two females. The age at BCPS ranged from 1 to 12 years (mean 4.3 +/- 3.9 years), and body weight varied between 8.2 and 29.4 kg (mean 13.8 +/- 7.8 kg). All six patients had an unbalanced complete atrioventricular septal defect, with right ventricular hypoplasia present in five and left ventricular hypoplasia in one.
There were no operative deaths, but one case required takedown of the BCPS. Except for this case, postoperative courses were generally uneventful. The median duration of follow-up was 46 months (range 12-80 months). Only two of five survivors after BCPS underwent a subsequent Fontan procedure, and one of these patients died of pulmonary hypertension post-operatively. The remaining three patients appeared to have significant risk factors for the Fontan procedure due to severe common atrioventricular valve regurgitation or persistent pulmonary vascular obstructive disease, including one who has completely dropped out from the Fontan track.
Down's syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down's syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully.
关于唐氏综合征合并功能性单心室患儿的恰当治疗，目前信息匮乏。我们报告了6例唐氏综合征合并功能性单心室患者行双向腔肺分流术的结果。
在1991年1月至2004年12月期间，我们在263例行双向腔肺分流术（BCPS）的患者中确定了6例唐氏综合征患者。其中男性4例，女性2例。行BCPS时的年龄为1至12岁（平均4.3±3.9岁），体重在8.2至29.4千克之间（平均13.8±7.8千克）。所有6例患者均有不平衡型完全性房室间隔缺损，5例右心室发育不全，1例左心室发育不全。
无手术死亡病例，但有1例需要拆除BCPS。除该病例外，术后病程一般平稳。中位随访时间为46个月（范围12 - 80个月）。BCPS术后的5名幸存者中只有2例随后接受了Fontan手术，其中1例术后死于肺动脉高压。其余3例患者因严重的共同房室瓣反流或持续性肺血管阻塞性疾病，似乎有进行Fontan手术的显著风险因素，其中1例已完全退出Fontan手术流程。
由于持续性肺动脉高压和气道阻塞，唐氏综合征是功能性单心室患者的一个风险因素。这些结果表明，唐氏综合征患者的单心室修复存在困难，Fontan手术的患者选择应谨慎进行。
