Delmo Walter Eva Maria B, Hübler Michael, Alexi-Meskishvili Vladimir, Miera Oliver, Weng Yuguo, Loforte Antonio, Berger Felix, Hetzer Roland
Department of Cardiovascular and Thoracic Surgery Deutsches Herzzentrum Berlin, Augustenburger Platz 1, Berlin, Germany.
J Card Surg. 2009 Jul-Aug;24(4):383-91. doi: 10.1111/j.1540-8191.2008.00759.x.
Surgical options for infants with hypoplastic left heart syndrome (HLHS) and/or its variants are cardiac transplantation or the heart-preserving staged palliation with Norwood operation,followed by a two-staged Fontan procedure. We describe our 17-year experience with staged palliation of HLHS and/or its variants.
Between December 1989 and December 2006, 64 patients with HLHS and/or its variants underwent a Norwood procedure (mean age/weight, 11.8+/-2.5 days/3.4 kg). Forty-four patients had classical HLHS. Twenty-eight percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 25 patients underwent a bidirectional Glenn procedure (stage II) and 11 patients a modified Fontan procedure (stage III). Others await stage II and/or stage III. The follow-up was 143.2 patient-years.
Including the learning curve, overall early mortality from 1989 to 1999 after the Norwood procedure was 39.06%. This decreased tremendously for the last seven years, and reduced to 12.8% in 2000 to 2003 until 0% in 2004 to 2006 (p < 0.005). The causes of mortality were sepsis, capillary leak,or heart failure. Three patients died between stages II and III. One patient underwent heart transplantation after the second stage because of heart failure. Among 34 Norwood survivors, four are slightly tachypneic from a mild pulmonary hyperperfusion; one presents symptoms of minimal brain disease.
This report identified an outcome improvement after staged palliation of HLHS, attributed to an increase in experience and expertise gained over time. Lower operative weight, ascending aortic size, prolonged duration of cardiopulmonary bypass, and hypothermic circulatory arrest were identified to significantly influence early mortality after the Norwood procedure.
患有左心发育不全综合征（HLHS）及其变体的婴儿的手术选择是心脏移植或采用诺伍德手术进行保留心脏的分期姑息治疗，随后进行两阶段的Fontan手术。我们描述了我们在HLHS及其变体的分期姑息治疗方面17年的经验。
在1989年12月至2006年12月期间，64例患有HLHS及其变体的患者接受了诺伍德手术（平均年龄/体重，11.8±2.5天/3.4千克）。44例患者患有典型的HLHS。28%的患者伴有先天性心脏、结构和遗传异常。随后，25例患者接受了双向格林手术（第二阶段），11例患者接受了改良Fontan手术（第三阶段）。其他患者等待第二阶段和/或第三阶段手术。随访时间为143.2患者年。
包括学习曲线在内，1989年至1999年诺伍德手术后的总体早期死亡率为39.06%。在过去七年中这一死亡率大幅下降，在2000年至2003年降至12.8%，到2004年至2006年降至0%（p<0.005）。死亡原因是败血症、毛细血管渗漏或心力衰竭。3例患者在第二阶段和第三阶段之间死亡。1例患者在第二阶段后因心力衰竭接受了心脏移植。在34例诺伍德手术幸存者中，4例因轻度肺血流灌注过多而轻度呼吸急促；1例有轻微脑部疾病症状。
本报告显示HLHS分期姑息治疗后的结果有所改善，这归因于随着时间推移经验和专业知识的增加。较低的手术体重、升主动脉大小、较长的体外循环时间和低温循环停止被确定为对诺伍德手术后的早期死亡率有显著影响。
