Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies
Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies
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DOI：
10.1093/ejcts/ezu547
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发表时间：
2015-11-01
影响因子：
3.4
通讯作者：
Kanter, Kirk
中科院分区：
文献类型：
--
作者：
Alsoufi, Bahaaldin;Deshpande, Shriprasad;Kanter, Kirk
目的：多阶段姑息治疗是左心发育不良综合征（HLHS）及相关单心室畸形患儿的主要治疗策略。如果这种缓解策略失败，则需要心脏移植（HT）。据报道，由于几个免疫、临床和解剖学风险因素，既往接受过诺伍德手术的儿童HT的结果较差。我们报告了我们的机构的结果后HT的儿童谁有一个先前的诺伍德operation.METHODS：在1994年和2013年之间，107先天性心脏病的儿童进行HT。我们研究了既往接受过诺伍德手术的儿童（n = 24）的早期和晚期结局，并分析了影响生存率的风险因素。生存率随后与对照组的83名儿童先天性心脏病没有以前的诺伍德手术谁收到HT。结果：24名儿童与以前的诺伍德手术进行HT。大多数（22/24，92%）患有HLHS。在诺伍德（n = 2，8.3%）、格伦（n = 17，70.8%）或丰坦（n = 5，20.8%）手术后列出了儿童。10例（42%）患者的群体反应性抗体（PRA）> 10%。入组时的中位年龄为2.7岁（范围0.4-16.8），HT时的中位年龄为3.0岁（范围0.6-16.8），中位等待时间为63.5天（范围1-554）。住院死亡率为1/24（4%）。1年、5年和10年的总体参数生存率估计值分别为85%、65%和52%。生存率不受列名状态、末次缓解期、年龄或高PRA的影响。影响生存率的唯一重要因素是我们研究中的晚期患者，其5年生存率显著上级晚期患者（100 vs 42%，P = 0.0003）。总生存率与未行诺伍德手术的先天性心脏病儿童对照组相当（10年时52% vs 53%，P = 0.97）。总体而言，24例患者中有3例需要再次移植，只有一个晚期survivor.CONCLUSIONS：儿童未能多级姑息HLHS可能需要HT，往往在格伦手术。本组患儿的HT结果与其他先天性心脏病患儿相当。后期移植前管理、免疫抑制和门诊治疗的改善可能使这一特别危险的患者群体受益。
OBJECTIVES: Multistage palliation is the mainstay management strategy of children with hypoplastic left heart syndrome (HLHS) and related single ventricle anomalies. If this palliation strategy fails, heart transplantation (HT) is required. The results of HT in children who had a prior Norwood operation are reportedly poor due to several immune, clinical and anatomical risk factors. We report our institutional outcomes following HT in children who had a prior Norwood operation.METHODS: Between 1994 and 2013, 107 children with congenital heart disease underwent HT. We examined early and late outcomes in our study cohort of children who had a prior Norwood operation (n = 24), and analysed risk factors affecting survival. Survival was subsequently compared with a control group of 83 children with congenital heart disease without a prior Norwood operation who received HT.RESULTS: Twenty-four children with a prior Norwood operation underwent HT. The majority (22/24, 92%) had HLHS. Children were listed following Norwood (n = 2, 8.3%), Glenn (n = 17, 70.8%) or Fontan (n = 5, 20.8%) operation. Ten (42%) patients had panel reactive antibodies (PRAs) > 10%. Median age at listing was 2.7 (range 0.4-16.8) years and median age at the time of HT was 3.0 (range 0.6-16.8) years, with the median waiting list duration of 63.5 (range 1-554) days. Hospital mortality was 1/24 (4%). Overall parametric survival estimates at 1, 5 and 10 years were 85, 65 and 52%. Survival was not affected by listing status, last palliation stage, age or high PRA. The only significant factor affecting survival was the later era in our series with significantly superior 5-year survival (100 vs 42%, P = 0.0003). Overall survival was comparable with the control group of children with congenital heart disease and no prior Norwood operation (52 vs 53% at 10 years, P = 0.97). Overall, 3 of 24 patients required retransplantation with only one late survivor.CONCLUSIONS: Children failing multistage palliation of HLHS may require HT, often following the Glenn operation. HT results in this group are comparable with those in other children with congenital heart disease. Improvements in pretransplant management, immune suppression and outpatient care in the later era might have specifically benefited this particularly risky group of patients.