Michielon Guido, DiSalvo Giovanni, Fraisse Alain, Carvalho Julene S, Krupickova Sylvia, Slavik Zdenek, Bartsota Margarita, Daubeney Pierce, Bautista Carles, Desai Ajay, Burmester Margarita, Macrae Duncan
Department of Congenital Heart Surgery, Royal Brompton Hospital, Imperial College, London, UK.
Department of Paediatric Cardiology, Royal Brompton Hospital, Imperial College, London, UK.
Eur J Cardiothorac Surg. 2020 Jun 1;57(6):1113-1121. doi: 10.1093/ejcts/ezaa074.
The interstage mortality rate after a Norwood stage 1 operation remains 12-20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome.
A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure.
Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood-Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock-Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan-Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan-Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant.
In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.
在目前的系列研究中，诺伍德一期手术后的过渡期死亡率仍为12%-20%。住院过渡期便于加强治疗，可能改善预后。
针对左心发育不全综合征（HLHS）及其变异型开展一项回顾性研究，在诺伍德手术后提供住院治疗直至二期手术完成。系统地进行每日和每周检查，包括二维和斑点追踪超声心动图检查。主要终点包括二期手术完成前的总生存率以及过渡期无需加强治疗。此外，我们计算了斑点追踪超声心动图心肌变形在预测诺伍德手术后死亡/移植方面的敏感性和特异性。
2015年至2019年期间，33例HLHS（24例）或HLHS变异型（9例）新生儿接受了诺伍德一期手术（31例）或杂交姑息治疗，随后进行了全面的二期手术（2例）。18例（54.5%）新生儿首选诺伍德-佐野一期手术；13例（39.4%）新生儿进行了经典的带Blalock-Taussig分流的诺伍德手术。诺伍德一期手术的30天死亡率为6.2%。诺伍德一期手术后实施了住院过渡期策略，过渡期死亡率为3.4%。诺伍德一期手术和过渡期的总Kaplan-Meier生存率为90.6±5.2%。5例（17.2%）患者在过渡期2.5±1.2个月时因房性心律失常（2例）、佐野分流狭窄（1例）和需要高频振荡器的肺炎（2例）而需要加强治疗；无死亡病例。27例患者在4.7±1.2个月时完成了双向格林手术（25例）或全面的诺伍德二期手术（2例），生存率为92.6%。4.3年（平均25.3±15.7个月）时的总体Kaplan-Meier生存率为80.9±7.0%。诺伍德一期手术后30天纵向应变下降8.7%对死亡/移植的敏感性为100%，特异性为81%。
住院过渡期便于加强治疗，这似乎对降低诺伍德手术过渡期死亡有效。诺伍德一期手术后纵向应变显著降低是预后不良的有力预测指标。
