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。
