Congenital Heart Surgeons' Society Data Center, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Division of Pediatric Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
J Thorac Cardiovasc Surg. 2017 Jan;153(1):163-172.e6. doi: 10.1016/j.jtcvs.2016.08.021. Epub 2016 Aug 31.
For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures.
In a critical LVOTO inception cohort (2005-2014; 20 institutions), a total of 564 neonates underwent stage 1 palliation with the Norwood operation with a modified Blalock-Taussig shunt (NW-BT; n = 232; 41%), Norwood operation with a right ventricle-to-pulmonary artery conduit (NW-RVPA; n = 222; 39%), or a hybrid procedure (n = 110; 20%). Post-stage 1 outcomes were analyzed via competing-risks and parametric hazard analyses and compared among all 564 patients and between patients who underwent propensity-matched hybrid and those who underwent NW-BT/NW-RVPA.
By 6 years after the stage 1 operation, 50% ± 3%, 7% ± 2%, and 4% ± 1% of patients transitioned to Fontan, transplantation, and biventricular repair, respectively, whereas 7% ± 2% were alive without transition and 32% ± 2% died. Risk factors for death without transition included procedure type, smaller ascending aorta, aortic valve atresia, and lower birth weight. Risk-adjusted 4-year survival was better after NW-RVPA than after NW-BT or hybrid (76% vs 60% vs 61%; P < .001). Furthermore, for neonates with lower birth weight (<∼2 kg), an interaction between birth weight and hybrid resulted in a trend toward better survival after hybrid compared with NW-BT or NW-RVPA. For propensity-matched neonates between hybrid and NW-BT (88 pairs), 4-year survival was similar (62% vs 57%; P = .58). For propensity-matched neonates between hybrid and NW-RVPA (81 pairs), 4-year survival was better after NW-RVPA (59% vs 75%; P = .008).
For neonates with critical LVOTO undergoing single-ventricle palliation, NW-RVPA was associated with the best overall survival. Hybrid strategies are not a lower-risk alternative to Norwood operations overall; however, the impact of lower birth weight on survival may be mitigated after hybrid procedures compared with Norwood operations.
对于患有严重左心室流出道梗阻（LVOTO）的新生儿，杂交手术是一期 Norwood 手术的替代方法。然而，尽管有优势，但结果并不明确。因此，我们比较了一期杂交与 Norwood 手术后的结果。
在一项严重 LVOTO 起始队列研究中（2005-2014 年；20 个机构），共有 564 例新生儿接受一期姑息治疗，包括 Norwood 手术伴改良 Blalock-Taussig 分流术（NW-BT；n=232；41%）、Norwood 手术伴右心室至肺动脉通道（NW-RVPA；n=222；39%）或杂交手术（n=110；20%）。通过竞争风险和参数风险分析分析一期手术后的结果，并在所有 564 例患者之间以及在接受倾向性匹配杂交与 NW-BT/NW-RVPA 的患者之间进行比较。
一期手术后 6 年时，分别有 50%±3%、7%±2%和 4%±1%的患者转为 Fontan 手术、移植和双心室修复，而 7%±2%的患者存活且无需转归，32%±2%的患者死亡。无转归的死亡风险因素包括手术类型、升主动脉较小、主动脉瓣闭锁和出生体重较低。风险调整后的 4 年生存率 NW-RVPA 优于 NW-BT 或杂交（76%比 60%比 61%；P<0.001）。此外，对于出生体重较低（<∼2kg）的新生儿，出生体重与杂交之间的相互作用导致与 NW-BT 或 NW-RVPA 相比，杂交后生存趋势更好。对于 NW-BT 与杂交的倾向性匹配新生儿（88 对），4 年生存率相似（62%比 57%；P=0.58）。对于 NW-RVPA 与杂交的倾向性匹配新生儿（81 对），NW-RVPA 后的 4 年生存率更好（59%比 75%；P=0.008）。
对于患有严重 LVOTO 的新生儿，接受单心室姑息治疗，NW-RVPA 与总体生存率最佳相关。杂交策略并非整体上比 Norwood 手术风险更低的替代方法；然而，与 Norwood 手术相比，杂交手术后的生存可能会因出生体重较低而得到缓解。
