Artrip John H, Campbell David N, Ivy D Dunbar, Almodovar Melvin C, Chan Kak-Chen, Mitchell Max B, Clarke David R, Lacour-Gayet François
The Children's Hospital Heart Institute, The Children's Hospital, Denver, Colorado 80218-1088, USA.
Ann Thorac Surg. 2006 Oct;82(4):1252-7; discussion 1258-9. doi: 10.1016/j.athoracsur.2006.04.062.
Classic options for treatment of hypoplastic left heart syndrome include the Norwood procedure (NW) and heart transplantation (HT). Recently off-pump palliative procedures were introduced in the management of these patients. Risk factors influencing the decision between the NW with staged reconstruction or off-pump palliation and HT were assessed.
Between January 2002 and January 2006, 69 patients with hypoplastic left heart syndrome were referred for either a NW (n = 33) or HT (n = 36). Patients referred for HT underwent off-pump palliation (catheter-based, n = 20, or surgical hybrid procedures, n = 7) until a donor organ became available: including patent ductus arteriosus stenting (80.6%), atrial septostomy (41.7%), and branch pulmonary artery banding (55.6%).
Heart transplantation patients were more complex, based on a higher Aristotle Comprehensive Complexity score calculated at birth (HT 18.8 +/- 2.4 versus NW 17.7 +/- 1.7; p < 0.05). Presurgical death was significantly greater for HT referrals (HT 27.8% versus NW 3.0%; p < 0.01); however, there was no difference between the two groups in operative (HT 11.5% versus NW 21.9%; not significant) or overall mortality (HT 36.1% versus NW 24.2%; not significant). No independent risk factors for death were identified with HT; however, a lower birth weight (<2.5 kg) and a higher Aristotle score (>20) correlated with surgical death with NW (p < 0.01). Noticeably, surgical survival was 85.7% for infants with birth weight of 2.5 kg or greater undergoing NW.
Overall survival is similar for hypoplastic left heart syndrome patients referred for the NW or HT. Lower birth weight and higher complexity are risk factors for patients undergoing NW. Off-pump palliation followed by HT or further staged palliation should be considered for these high-risk hypoplastic left heart syndrome patients.
治疗左心发育不全综合征的传统方法包括诺伍德手术（NW）和心脏移植（HT）。最近，非体外循环姑息手术被引入这些患者的治疗中。评估了影响在NW分期重建或非体外循环姑息治疗与HT之间做出决策的危险因素。
2002年1月至2006年1月期间，69例左心发育不全综合征患者被转诊接受NW（n = 33）或HT（n = 36）治疗。转诊接受HT的患者在获得供体器官之前接受非体外循环姑息治疗（基于导管的，n = 20，或外科杂交手术，n = 7）：包括动脉导管未闭支架置入术（80.6%）、房间隔造口术（41.7%）和分支肺动脉环扎术（55.6%）。
根据出生时计算的较高的亚里士多德综合复杂性评分，心脏移植患者情况更复杂（HT 18.8 ± 2.4对NW 17.7 ± 1.7；p < 0.05）。转诊接受HT的患者术前死亡率显著更高（HT 27.8%对NW 3.0%；p < 0.01）；然而，两组在手术死亡率（HT 11.5%对NW 21.9%；无显著差异）或总体死亡率（HT 36.1%对NW 24.2%；无显著差异）方面没有差异。未发现HT患者死亡的独立危险因素；然而，较低的出生体重（<2.5 kg）和较高的亚里士多德评分（>20）与NW手术死亡相关（p < 0.01）。值得注意的是，出生体重2.5 kg或更大的婴儿接受NW手术的生存率为85.7%。
转诊接受NW或HT的左心发育不全综合征患者的总体生存率相似。较低的出生体重和较高的复杂性是接受NW手术患者的危险因素。对于这些高危左心发育不全综合征患者，应考虑非体外循环姑息治疗后进行HT或进一步的分期姑息治疗。
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