Pontailler Margaux, Gaudin Régis, Lenoir Marien, Haydar Ayman, Kraiche Diala, Bonnet Damien, Vouhé Pascal, Raisky Olivier
Department of Pediatric Cardiac Surgery, University Paris Descartes and Necker Sick Children Hospital, Paris, France.
Department of Pediatric Cardiology, University Paris Descartes and Necker Sick Children Hospital, Paris, France.
Eur J Cardiothorac Surg. 2017 May 1;51(5):1003-1008. doi: 10.1093/ejcts/ezx021.
We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach.
This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia. Initial results led to tightening of the pulmonary artery band from 3.5 mm to 2.5 mm.
From July 2014 to May 2016, 15 patients were initially palliated (13 patients with aortic atresia/mitral atresia, 1 with double inlet right ventricle, 1 with tricuspid atresia) at a median age of 5 days (1-8 days) and a median weight of 3.0 kg (2.3-3.9 kg). All but 2 patients were discharged from the hospital: There was 1 early in-hospital death and 1 patient required hospitalization in the intensive care unit until stage 2. There were also 4 interstage deaths. Nine patients underwent Stage 2 and 1 patient is still waiting. Compared to the classic Norwood procedure, early mortality decreased significantly (43.3-6.7%; P = 0.0074) and overall mortality before Stage 2 was halved (61.6-33.3%). Postoperative morbidity was also reduced.
This new surgical palliative approach to hypoplastic left heart syndrome, particularly useable technically, seems to combine the advantages of the hybrid procedure by avoiding cardiac ischaemia and ventriculotomy without the complications of PDA stenting and restrictive atrial septectomy. Although the mortality rate decreased significantly, it remains substantial in small-volume centres, especially in the interstage period.
在一项前瞻性研究中，我们描述了一种受杂交诺伍德手术启发的用于治疗左心发育不全综合征的新型手术技术。
这种新的新生儿姑息手术包括在体外循环且不进行主动脉阻断和心脏停搏的情况下，用同种异体肺动脉移植片替换动脉导管未闭（PDA）并进行主动脉弓成形术，同时对双侧肺动脉进行束带术和房间隔切除术。初步结果导致肺动脉束带从3.5毫米收紧至2.5毫米。
2014年7月至2016年5月，15例患者接受了初始姑息治疗（13例主动脉闭锁/二尖瓣闭锁、1例右心室双入口、1例三尖瓣闭锁），中位年龄5天（1 - 8天），中位体重3.0千克（2.3 - 3.9千克）。除2例患者外，其他所有患者均出院：有1例早期院内死亡，1例患者在重症监护病房住院直至二期手术。还有4例过渡期死亡。9例患者接受了二期手术，1例患者仍在等待。与经典诺伍德手术相比，早期死亡率显著降低（43.3% - 6.7%；P = 0.0074），二期手术前的总体死亡率减半（61.6% - 33.3%）。术后发病率也有所降低。
这种治疗左心发育不全综合征的新手术姑息方法，特别是在技术上易于应用，似乎结合了杂交手术的优点，避免了心肌缺血和心室切开术，同时没有PDA支架置入和限制性房间隔切除术的并发症。尽管死亡率显著降低，但在小容量中心仍然很高，尤其是在过渡期。
