Division of Pediatric Cardiac Surgery, Columbia University College of Physicians and Surgeons, New York, New York.
Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York.
Ann Thorac Surg. 2021 Dec;112(6):2012-2019. doi: 10.1016/j.athoracsur.2020.10.014. Epub 2020 Nov 6.
Patients with trisomy 21 (T21) often have elevated pulmonary vascular resistance, which may result in a suboptimal cavopulmonary connection (CPC) after a Glenn or Fontan operation. The purpose of this study was to assess, in a nationwide, multiinstitution cohort of patients with CPC, the impact of T21 on patient morbidity, mortality, and resource use.
A total of 23,271 pediatric patients with CPC (2004 to 2019) at 50 US hospitals were evaluated using the Pediatric Health Information System database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between Down syndrome and other risk factors and postoperative measures of morbidity, mortality, lengths of stay, and cost of hospitalization.
The overall prevalence of T21 among patients who had undergone Glenn and Fontan procedures was 1.5% (199 of 13,268) and 0.8% (78 of 1003), respectively. Among both CPC cohorts, T21 status significantly increased unadjusted mortality, hospital lengths of stay, and total costs of hospitalization compared with the non-T21 CPC cohort (all P < .001). Patients with T21 also had a higher incidence of prolonged mechanical ventilation compared with patients without T21 in both Glenn and Fontan groups (P < .001). Multivariable regression analysis further estimated that patients with T21 are associated with a 5.5-fold increase in mortality (P < .001) compared with patients without T21. Finally, patients with T21 had increased long-term mortality compared with their peers.
T21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality, and resource use after cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level and center-specific risk factors to improve outcomes for patients with T21.
21 三体综合征（T21）患者的肺血管阻力通常较高，这可能导致 Glenn 或 Fontan 手术后腔静脉-肺动脉吻合术（CPC）效果不佳。本研究的目的是在全国范围内的 CPC 多机构患者队列中评估 T21 对患者发病率、死亡率和资源利用的影响。
使用儿科健康信息系统数据库评估了 50 家美国医院的 23271 例 CPC 患儿（2004 年至 2019 年）。使用单变量和多变量回归分析评估了唐氏综合征和其他危险因素与术后发病率、死亡率、住院时间和住院费用之间的风险调整关联。
行 Glenn 和 Fontan 手术的患者中 T21 的总体患病率分别为 1.5%（13268 例中的 199 例）和 0.8%（1003 例中的 78 例）。在 CPC 两组中，T21 状态与非 T21 CPC 组相比，未调整死亡率、住院时间和住院总费用均显著增加（均 P <.001）。与非 T21 组相比，T21 组患者在行 Glenn 和 Fontan 手术的患者中，机械通气时间延长的发生率也更高（均 P <.001）。多变量回归分析进一步估计，与非 T21 患者相比，T21 患者的死亡率增加了 5.5 倍（P <.001）。最后，与非 T21 患者相比，T21 患者的长期死亡率增加。
T21 显著增加 CPC 后风险调整发病率、住院死亡率、长期死亡率和资源利用。需要进一步研究以明确可改变的患者水平和中心特异性危险因素，以改善 T21 患者的结局。
