Erek Ersin, Aydın Selim, Temur Bahar, Önalan Mehmet Akif, Suzan Dilek, İyigün Müzeyyen, Demir İbrahim Halil, Ödemiş Ender
Department of Cardiovascular Surgery, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Istanbul, Turkey.
Department of Cardiovascular Surgery, Marie Lannelongue Hospital, Paris, France.
Turk Gogus Kalp Damar Cerrahisi Derg. 2020 Apr 22;28(2):282-293. doi: 10.5606/tgkdc.dergisi.2020.18605. eCollection 2019 Jun.
In this study, we present the outcomes of hybrid and Norwood Stage I procedures for the treatment of hypoplastic left heart syndrome and its variants.
In this study, a total of 97 pediatric patients who were operated due to hypoplastic left heart syndrome and its variants between March 2011 and October 2018 were retrospectively analyzed. Thirty-two of the patients (28 males, 4 females; median age 5 days; range, 1 to 25 days) underwent Norwood Stage I operation (Group N), while the remaining 65 patients (44 males, 21 females; median age 6 days; range, 1 to 55 days) underwent a hybrid procedure (Group H). Both treatment strategies were compared.
The median body weight in Group H was significantly lower and the number of patients with a low birth weight (<2,500 g) was significantly higher than Group N (p=0.002 and 0.004, respectively). The postoperative early mortality rate was similar between the groups. Univariate and multivariate analyses revealed that the need for preoperative mechanical ventilation was a significant factor for mortality (p=0.004 and 0.003, respectively). Syndromic appearance was also a significant factor the multivariate analysis (p=0.03). There was a statistically significant difference between the groups in terms of the inter-stage mortality rates (p=0.0045). Second-stage procedure was performed in 32 patients. The early mortality rate after the Glenn operation was 7.6%. Six patients died after comprehensive Stage II operation. Five patients underwent biventricular repair and 8 patients had third-stage fenestrated extracardiac Fontan operation (Group N, n=7 and Group H, n=1). The Kaplan-Meier survival curve demonstrated that Group N had a higher survival rate at both one and five years than Group H, although the difference was not statistically significant (p=0.15). Subgroup analysis showed that the Norwood procedure with Sano modification had the highest survival rate with 40% at five years.
Our study results show that patients undergoing the Norwood procedure have a more uneventful course of inter-stage period and Stage II and III, despite drawbacks early after Stage I procedure. Based on our experiences, we recommend performing the hybrid intervention in patients with a poor clinical condition and a body weight of <2,500 g.
在本研究中，我们展示了用于治疗左心发育不全综合征及其变异型的杂交手术和诺伍德一期手术的结果。
本研究对2011年3月至2018年10月期间因左心发育不全综合征及其变异型接受手术的97例儿科患者进行了回顾性分析。其中32例患者（28例男性，4例女性；中位年龄5天；范围1至25天）接受了诺伍德一期手术（N组），其余65例患者（44例男性，21例女性；中位年龄6天；范围1至55天）接受了杂交手术（H组）。对两种治疗策略进行了比较。
H组的中位体重显著低于N组，低出生体重（<2500 g）患者的数量显著高于N组（分别为p = 0.002和0.004）。两组术后早期死亡率相似。单因素和多因素分析显示，术前需要机械通气是死亡率的一个重要因素（分别为p = 0.004和0.003）。综合征表现也是多因素分析中的一个重要因素（p = 0.03）。两组间阶段间死亡率存在统计学显著差异（p = 0.0045）。32例患者进行了二期手术。格林手术术后早期死亡率为7.6%。6例患者在二期综合手术后死亡。5例患者接受了双心室修复，8例患者进行了三期开窗体外循环Fontan手术（N组，n = 7；H组，n = 1）。Kaplan-Meier生存曲线显示N组在1年和5年时的生存率均高于H组，尽管差异无统计学意义（p = 0.1
