Division of Pediatric Cardiology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
Division of Pediatric Cardiology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
Ann Thorac Surg. 2018 Aug;106(2):595-601. doi: 10.1016/j.athoracsur.2018.03.007. Epub 2018 Apr 6.
Hypoplastic left heart syndrome and other cardiac lesions with ductal-dependent systemic circulation continue to be challenging to manage, especially in high-risk (HR) populations (those with prematurity, multiple congenital anomalies, moderate to severe tricuspid regurgitation, hemodynamic instability, intact atrial septum).
A retrospective study on our institution's experience implementing a hybrid strategy as initial palliation in HR patients with ductal-dependent systemic circulation in HR patients undergoing Norwood versus hybrid procedure. From July 2004 to May 2008, 16 HR patients underwent stage I Norwood procedure. After implementation of a hybrid strategy in 2008, 24 HR patients underwent hybrid procedure from May 2008 to November 2015.
There was no difference in gestational age, age at procedure, or hospital length of stay. The HR Hybrid group had lower mean weight (2.6 kg vs 3.1 kg, p = 0.026). Thirty-day mortality was lower in the HR Hybrid group (4% vs 31%, p = 0.019), although there was no difference in interstage mortality (17% vs 9%, p = 0.396). Catheter-based reintervention was more prevalent in the HR Hybrid group, but did not have a negative impact on survival. One-year transplant-free survival was similar (p = 0.416). HR Hybrid patients weighing less than 2.6 kg had higher overall survival (83% vs 25%, p = 0.013), as did patients who were premature (70% vs 0%, p = 0.003).
In high-risk patients, the hybrid procedure appears to have lower 30-day mortality and may have a survival benefit in premature patients and those less than 2.6 kg. Long-term attrition in this high-risk population is ongoing regardless of early strategy.
患有左心发育不全综合征和其他依赖导管循环系统的心脏病变的患者仍然难以治疗，尤其是在高危（HR）人群（早产儿、多发先天性畸形、中重度三尖瓣反流、血流动力学不稳定、房间隔完整）中。
对我院实施的 HR 患者合并依赖导管循环系统的左心发育不全综合征患者的混合策略作为初始姑息治疗的经验进行回顾性研究，该 HR 患者接受了 Norwood 手术与混合手术的比较。2004 年 7 月至 2008 年 5 月，16 例 HR 患者接受了 I 期 Norwood 手术。2008 年实施混合策略后，2008 年 5 月至 2015 年 11 月，24 例 HR 患者接受了混合手术。
两组患者的胎龄、手术年龄或住院时间均无差异。HR 混合组的平均体重较低（2.6kg 比 3.1kg，p=0.026）。HR 混合组的 30 天死亡率较低（4%比 31%，p=0.019），尽管间期中死亡率无差异（17%比 9%，p=0.396）。HR 混合组经导管再次介入更为普遍，但并未对生存率产生负面影响。1 年无移植生存率相似（p=0.416）。体重低于 2.6kg 的 HR 混合组患者的总生存率较高（83%比 25%，p=0.013），早产儿（70%比 0%，p=0.003）的生存率也较高。
在高危患者中，混合手术的 30 天死亡率似乎较低，在早产儿和体重低于 2.6kg 的患者中可能具有生存优势。无论早期策略如何，高危人群的长期流失仍在继续。
