Heart Institute, Cincinnati Children's Hospital, Cincinnati, Ohio.
Department of Cardiothoracic Surgery, Northwell Health, Manhasset, New York.
Pediatr Transplant. 2020 May;24(3):e13682. doi: 10.1111/petr.13682. Epub 2020 Feb 17.
Pediatric heart-lung transplantation (HLT) is rare, and no report has analyzed patient outcomes since time of listing. We analyzed pediatric HLTs to understand risk factors for waitlist and post-HLT mortality.
All pediatric (<18 year old) HLT candidates were identified within the UNOS database (n = 573) and grouped by age, era, and by diagnosis. Logistical regression and Cox proportional hazard modeling identified risk factors for 6-month WL and overall post-transplant mortality.
209/573 (37%) HLT candidates were transplanted, 7% recovered, 42% died waiting, and 15% were removed for another/unknown reason. Diagnoses were primary pulmonary hypertension(n = 130), congenital heart disease(CHD) without Eisenmenger's syndrome (ES) (n = 65), CHD with ES (n = 73), and other (n = 305). Patients with a diagnosis other than CHD with ES (OR: 7.55, P = .001), on IV inotropic support (OR: 2.79, P < .001), and infants (OR: 2.20, P = .004) were associated with waitlist mortality. There has been a 56% reduction in HLTs across eras (Era 1:10.8/yr vs Era 2:4.7/yr). Risk factors for post-transplant mortality were ECMO (HR: 4.1, P = .016), and being infant (HR: 2.2, P = .04) or 1-11 years old (HR: 1.78, P = .015). ECMO patients have an 87% 2-year mortality rate with a median post-transplant survival of 64 days. Overall, post-transplant survival was unchanged (log-rank P = .067) between eras. Excluding ECMO patients, in the recent era 29 non-infant patients with primary pulmonary hypertension had 93% 1-year survival and 67% 5-year survival.
Nearly 600 pediatric patients have been listed for HLT in UNOS, although numbers are decreasing in the current era. HLT for a patient on ECMO appears to be an ineffective strategy; however, in well-selected cohorts, HLT can provide considerable post-transplant survival.
儿科心肺移植(HLT)较为罕见,且尚无研究分析患者在等待名单上的预后情况。本研究分析儿科 HLT 以明确等待名单和 HLT 后死亡率的相关危险因素。
我们在 UNOS 数据库中(n=573)识别所有儿科(<18 岁)HLT 候选者,并根据年龄、时代和诊断进行分组。Logistic 回归和 Cox 比例风险模型确定 6 个月 WL 和整体移植后死亡率的危险因素。
209/573(37%)HLT 候选者接受了移植,7%的患者恢复,42%等待期间死亡,15%因其他/未知原因被移除。诊断为原发性肺动脉高压(n=130)、无艾森曼格综合征(ES)的先天性心脏病(CHD)(n=65)、有 ES 的 CHD(n=73)和其他(n=305)。非有 ES 的 CHD 患者(OR:7.55,P=0.001)、正在使用静脉内正性肌力支持(OR:2.79,P<0.001)和婴儿(OR:2.20,P=0.004)与等待名单上的死亡率相关。不同时代 HLT 数量减少了 56%(时代 1:10.8/年 vs 时代 2:4.7/年)。移植后死亡率的危险因素为 ECMO(HR:4.1,P=0.016)和婴儿(HR:2.2,P=0.04)或 1-11 岁(HR:1.78,P=0.015)。ECMO 患者的 2 年死亡率为 87%,中位移植后生存期为 64 天。总体而言,时代之间的移植后生存率没有变化(对数秩 P=0.067)。排除 ECMO 患者后,在最近的时代 2 中,93%的 29 例原发性肺动脉高压非婴儿患者 1 年生存率为 67%,5 年生存率为 67%。
在 UNOS 中,近 600 名儿科患者被列入 HLT 名单,尽管目前这一数字正在减少。对于正在使用 ECMO 的患者进行 HLT 似乎不是一种有效的策略;然而,在经过精心选择的患者中,HLT 可以提供相当可观的移植后生存率。