Takai Akari, Yamagishi Masaaki, Ikeda Kazuyuki, Sugimoto Atsuya, Ichise Eisuke, Maeda Yoshinobu, Teramukai Satoshi, Hasegawa Tatsuji, Oda Shinichiro, Iehara Tomoko
Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
Department of Pediatrics, Hananoki Medical Welfare Center, Kyoto, Japan.
Am J Med Genet A. 2023 Nov;191(11):2703-2710. doi: 10.1002/ajmg.a.63401. Epub 2023 Sep 12.
Congenital heart disease (CHD) is common among patients with trisomy 18 (T18), but cardiac surgery has been rarely indicated for T18 patients due to their short life span. Although the therapeutic effects of aggressive interventions were recently demonstrated for T18 patients, the subjects and factors examined varied, resulting in inconsistent findings. Therefore, the effects of cardiac surgery for T18 remain unclear. We herein investigated the outcomes of cardiac palliative surgery for CHD with increased pulmonary blood flow in T18 patients. 27 patients were examined: 13 (48.1%) underwent cardiac palliative surgery and 14 (51.9%) did not. Median survival times in the no-surgery and surgery groups were 223.0 days (95% confidence interval [CI]: 46-361 days) and 723.0 days (95% CI: 360-1447 days), respectively. The number of patients with pulmonary hypertension significantly differed between the two groups (5 of 14 in the no-surgery group and 0 in the surgery group). Five of 14 patients in the no-surgery group and 10 of 13 in the surgery group were discharged to home care (odds ratio: 10.8 [95% CI: 1.07-110.0]). Therefore, cardiac palliative surgery may be used to treat CHD with increased pulmonary blood flow in T18 patients.
先天性心脏病（CHD）在18三体综合征（T18）患者中很常见，但由于T18患者寿命较短，很少对其进行心脏手术。尽管最近已证明积极干预对T18患者有治疗效果，但所研究的对象和因素各不相同，结果也不一致。因此，心脏手术对T18患者的影响仍不明确。我们在此研究了T18患者中因肺血流量增加而接受心脏姑息手术治疗CHD的结果。共检查了27例患者：13例（48.1%）接受了心脏姑息手术，14例（51.9%）未接受手术。非手术组和手术组的中位生存时间分别为223.0天（95%置信区间[CI]：46 - 361天）和723.0天（95%CI：360 - 1447天）。两组间肺动脉高压患者数量有显著差异（非手术组14例中有5例，手术组为0例）。非手术组14例患者中有5例、手术组13例中有10例出院接受家庭护理（比值比：10.8[95%CI：1.07 - 110.0]）。因此，心脏姑息手术可用于治疗T18患者中因肺血流量增加而导致的CHD。
