Hartyánszky I, Dobos M, Szabolcs J, Mihályi S, Lozsádi K, Fekete G
Gottsegen György Országos Kardiológiai Intézet Csecsemó- és Gyermekszívsebészeti Osztály.
Orv Hetil. 2000 Sep 24;141(39):2119-22.
Authors analysed 359 cases with Down's syndrome and congenital heart defects registered between 1974-1997 in Hungary. The total death rate was 19.9% (70 cases). Mortality in the operated group (85 cases) was 10.5% (9 patients), in the non-operated group (274 cases) 22.2% (61 patients). The death rate was lower in the group with early primary reconstruction (2.3%) than in the group with palliation + reconstruction (15.3%), or in the group with only palliative procedure (20%). These results indicate that the life expectancy of infants and children with Down's syndrome and congenital heart disease after early primary reconstructive procedure is the same as in Down syndrome patients without cardiac defects. The prognosis depends on the patient's social circumstances. The results after correct surgical procedure in patients with the same cardiac defect are similar to that of the patients with or without Down's syndrome.
作者分析了1974年至1997年间在匈牙利登记的359例唐氏综合征合并先天性心脏病病例。总死亡率为19.9%(70例)。手术组(85例)的死亡率为10.5%(9例患者),非手术组(274例)的死亡率为22.2%(61例患者)。早期一期重建组的死亡率(2.3%)低于姑息治疗+重建组(15.3%)或仅接受姑息治疗组(20%)。这些结果表明,唐氏综合征合并先天性心脏病的婴幼儿在接受早期一期重建手术后的预期寿命与无心脏缺陷的唐氏综合征患者相同。预后取决于患者的社会环境。患有相同心脏缺陷的患者在接受正确手术后的结果与患有或不患有唐氏综合征的患者相似。