Division of Paediatric Cardiology, University Children's Hospital Zurich, Zurich, Switzerland.
Eur J Cardiothorac Surg. 2012 Jul;42(1):33-9. doi: 10.1093/ejcts/ezr286. Epub 2012 Jan 18.
Neonates with hypoplastic left heart syndrome (HLHS) are at risk of high mortality and neurodevelopmental morbidity. As an alternative to Norwood-type stage I palliation, the hybrid procedure has been developed. It consists of bilateral pulmonary artery banding, catheter-based stenting of the arterial duct and balloon atrioseptostomy and delays open-heart surgery. Thus, it may be associated with a better outcome. The aim of this study was to determine the mortality and neurodevelopmental outcome in patients with HLHS and other univentricular heart (UVH) defects treated with hybrid or Norwood procedures.
Thirty-one children (18 males) with HLHS and other UVH defects undergoing Norwood or hybrid procedure between 2004 and 2008 were consecutively enrolled. Mortality and neurodevelopmental outcome at 1 year of age were determined.
One-year mortality was 36% (31% in the hybrid vs. 39% in the Norwood group, P=0.71). Predictors of mortality were lower birth weight (P=0.02), older age at first procedure (P=0.02) and smaller size of ascending aorta (P=0.05). Overall, median psychomotor development index (PDI) and mental development index (MDI) of the Bayley Scales of Infant Development II were lower than the norm of 100 [PDI 57 (49-99), P<0.001; MDI 91 (65-109), P=0.002]. No effect of surgical treatment on neurodevelopmental outcome was found. Predictors of impaired motor outcome were length of hospital stay (LOHS) (P=0.01), lower body weight at second procedure (P=0.004) and female sex (P=0.01). Predictors of impaired cognitive outcome were longer mechanical ventilation time (P=0.03), intensive care unit stay (P=0.04) and LOHS (P<0.001), respectively.
Mortality at 1 year of age is comparable between patients undergoing hybrid and Norwood procedures. Early neurodevelopmental outcome is significantly impaired in patients with both HLHS and other UVH defects. Multicentre randomized studies are needed to determine the long-term neurodevelopmental outcome of children treated with the hybrid procedure.
患有左心发育不全综合征（HLHS）的新生儿死亡率高，神经发育不良。作为 Norwood Ⅰ期姑息性手术的替代方法，已经开发出杂交手术。它包括双侧肺动脉带，导管动脉导管支架和球囊房间隔造口术，并延迟了心脏直视手术。因此，它可能与更好的结果相关。本研究的目的是确定接受杂交或 Norwood 手术治疗的 HLHS 和其他单心室心脏（UVH）缺陷患者的死亡率和神经发育结局。
2004 年至 2008 年间，连续纳入 31 名（男 18 名）接受 Norwood 或杂交手术的 HLHS 和其他 UVH 缺陷患儿。确定了 1 岁时的死亡率和神经发育结局。
1 年死亡率为 36%（杂交组为 31%，Norwood 组为 39%，P=0.71）。死亡率的预测因素是出生体重较低（P=0.02），初次手术年龄较大（P=0.02）和升主动脉较小（P=0.05）。总体而言，Bayley 婴儿发育量表 II 的精神运动发育指数（PDI）和智力发育指数（MDI）中位数均低于 100 的正常值[PDI 57（49-99），P<0.001；MDI 91（65-109），P=0.002]。未发现手术治疗对神经发育结局有影响。运动结局受损的预测因素是住院时间（LOHS）（P=0.01），第二次手术时体重较低（P=0.004）和女性（P=0.01）。认知结局受损的预测因素分别为机械通气时间较长（P=0.03），重症监护病房停留时间（P=0.04）和 LOHS（P<0.001）。
1 岁时的死亡率在接受杂交和 Norwood 手术的患者之间相似。HLHS 和其他 UVH 缺陷患者的早期神经发育结局明显受损。需要多中心随机研究来确定接受杂交手术治疗的儿童的长期神经发育结局。
