左心发育不良综合征患儿行 Norwood 分流术对心脏移植的影响：多中心研究。
Impact of initial Norwood shunt type on young hypoplastic left heart syndrome patients listed for heart transplant: A multi-institutional study.
机构信息
Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama.
Pediatric Cardiology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
出版信息
J Heart Lung Transplant. 2016 Mar;35(3):301-305. doi: 10.1016/j.healun.2015.10.032. Epub 2015 Oct 30.
BACKGROUND
Pulmonary blood flow during Stage 1 (Norwood) palliation for hypoplastic left heart syndrome (HLHS) is achieved via modified Blalock-Taussig shunt (MBT) or right ventricle to pulmonary artery conduit (RVPA). Controversy exists regarding the differential impact of shunt type on outcome among those who require transplantation early in life. In this study we explored waitlist and post-transplant outcomes within this sub-population stratified by shunt type.
METHODS
Eligible patients were enrolled through the Pediatric Heart Transplant Study (PHTS) database. Patients included those listed for heart transplantation at 1 of 35 participating centers, all of whom were <6 years of age and with a diagnosis of HLHS (and variants) status post Stage 1 palliation with MBT or RVPA. Standard risk factors for death were analyzed using multivariable hazards modeling.
RESULTS
Between 2010 and 2013, 190 patients were identified. Compared with the RVPA group (n = 111), the MBT group (n = 79) was less likely to have undergone a Glenn palliation (41% vs 73%, p < 0.001), were younger at listing (median age 1.3 vs 1.8 years, p = 0.05), had lower median weight (7.9 vs 9.4 kg, p = 0.02), and were more likely to be mechanically ventilated at listing (35% vs 22%, p = 0.04). There were no significant differences in median waitlist time (1.7 vs 2.6 months, p = 0.2) or rate of transplantation (61% vs 60%, p = 1.0). Among waitlisted patients, 3-month survival was less for MBT compared with RVPA patients (74% vs 91%, p = 0.02). Patients who had not yet achieved Glenn palliation before listing had lower waitlist 3-month survival (76% vs 90%, p = 0.02). In MBT infants <1 year old, there was a trend toward improved survival in those with Glenn palliation compared to those without (100% vs 68%, p = 0.08). Early post-transplant mortality rates were similar between the RVPA and MBT groups (p = 0.4) with overall survival 84% at 1 year.
CONCLUSIONS
Among HLHS patients, the need for transplant before Glenn palliation is associated with poorer waitlist survival. Waitlist survival is poorer in the MBT group, with this difference driven by pre-Glenn MBT infants. Post-transplant outcomes were unaffected by shunt type.
背景
在左心发育不全综合征（HLHS）的 1 期（诺伍德）姑息治疗中，肺血流量通过改良的 Blalock-Taussig 分流术（MBT）或右心室至肺动脉导管（RVPA）来实现。对于那些在早期需要进行移植的患者，分流类型对结果的影响存在争议。在这项研究中，我们通过分流类型分层，在这个亚群中探讨了等待名单和移植后的结果。
方法
通过儿科心脏移植研究（PHTS）数据库纳入符合条件的患者。患者包括在 35 个参与中心中的 1 个接受心脏移植的患者，所有患者年龄均<6 岁，且在接受 MBT 或 RVPA 的 1 期姑息治疗后诊断为 HLHS（及变体）。使用多变量风险建模分析死亡的标准风险因素。
结果
在 2010 年至 2013 年间，共确定了 190 名患者。与 RVPA 组（n = 111）相比，MBT 组（n = 79）不太可能接受 Glenn 姑息治疗（41% vs 73%，p < 0.001），在列入名单时年龄较小（中位年龄 1.3 岁 vs 1.8 岁，p = 0.05），体重中位数较低（7.9 公斤 vs 9.4 公斤，p = 0.02），在列入名单时更有可能接受机械通气（35% vs 22%，p = 0.04）。中位等待名单时间（1.7 个月 vs 2.6 个月，p = 0.2）或移植率（61% vs 60%，p = 1.0）均无显著差异。在等待名单上的患者中，MBT 患者的 3 个月生存率低于 RVPA 患者（74% vs 91%，p = 0.02）。在列入名单前尚未进行 Glenn 姑息治疗的患者，3 个月的等待名单生存率较低（76% vs 90%，p = 0.02）。在 MBT 年龄<1 岁的婴儿中，与无 Glenn 姑息治疗的婴儿相比，有 Glenn 姑息治疗的婴儿的生存趋势更好（100% vs 68%，p = 0.08）。RVPA 和 MBT 组之间的早期移植后死亡率相似（p = 0.4），1 年总生存率为 84%。
结论
在 HLHS 患者中，在 Glenn 姑息治疗前需要进行移植与较差的等待名单生存率相关。MBT 组的等待名单生存率较差，这一差异是由 Glenn 前 MBT 婴儿驱动的。移植后的结果不受分流类型的影响。
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