Bove E L
Division of Pediatric Cardiovascular Surgery, University of Michigan Health System, Ann Arbor, USA.
Jpn J Thorac Cardiovasc Surg. 1999 Feb;47(2):47-56. doi: 10.1007/BF03217941.
Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically improved with either a protocol of staged reconstruction or cardiac transplantation. Currently, a significant shortage of suitable donor hearts restricts the applicability of transplantation for most newborns. At the University of Michigan, we have adopted a policy of staged reconstruction for all patients with hypoplastic left heart syndrome, reserving transplantation only for those unsuitable for reconstructive techniques. Between January 1990 and September 1998, 303 patients underwent the Norwood operation for classic hypoplastic left heart syndrome with an overall hospital survival of 76%. Among patients considered at standard risk, survival was significantly higher (86%) than that for those patients with important risk factors (42%), p = 0.0001. Adverse survival was most strongly associated with significant associated noncardiac congenital conditions (p = 0.008) and severe preoperative obstruction to pulmonary venous return (p = 0.03). Survival following second stage reconstruction with a hemi-Fontan or bidirectional Glenn procedure was 98%. The Fontan procedure has been completed in 117 of these patients with a hospital survival rate of 91%. Survival after the Fontan procedure improved significantly when the second stage of the reconstruction was completed with a hemi-Fontan procedure compared to a bidirectional Glenn, 98% vs 81%, p < .05. Among the patients considered at standard risk, actuarial survival was 70% at 5 years. The largest decrease in survival occurred in the first month of life and late deaths affected primarily those patients in the high risk group. Neurodevelopmental outcome studies demonstrated normal verbal and performance scores in the majority of patients. Among centers utilizing a protocol of transplantation, donor organ shortages have resulted in a mortality of approximately 25% while awaiting transplantation with 5 year survival rates for those actually receiving organs essentially equal to those for staged reconstruction. Staged reconstruction and transplantation have significantly improved the intermediate-term outlook for patients with hypoplastic left heart syndrome. Factors addressing improvements in early first stage survival following the Norwood would be expected to add significantly to an overall improved late outcome. Outcome following cardiac transplantation is limited by donor availability in addition to the late complications of infection, rejection, graft atherosclerosis, and lymphoproliferative disease.
曾经被认为是一种必死无疑的病症，对于患有左心发育不全综合征的新生儿，采用分期重建方案或心脏移植后，其预后已得到显著改善。目前，合适供体心脏的严重短缺限制了移植手术对大多数新生儿的适用性。在密歇根大学，我们对所有患有左心发育不全综合征的患者都采取了分期重建政策，仅对那些不适合重建技术的患者进行移植。在1990年1月至1998年9月期间，303例患者接受了针对典型左心发育不全综合征的诺伍德手术，总体住院生存率为76%。在被认为处于标准风险的患者中，生存率显著更高（86%），高于那些具有重要风险因素的患者（42%），p = 0.0001。不良生存与显著的相关非心脏先天性疾病（p = 0.008）和严重的术前肺静脉回流梗阻（p = 0.03）最为密切相关。采用半Fontan或双向格林手术进行二期重建后的生存率为98%。在这些患者中，已有117例完成了Fontan手术，住院生存率为91%。与双向格林手术相比，当二期重建采用半Fontan手术完成时，Fontan手术后的生存率显著提高，分别为98%和81%，p < 0.05。在被认为处于标准风险的患者中，5年精算生存率为70%。生存率下降最大的时期是在出生后的第一个月，晚期死亡主要影响高危组的患者。神经发育结局研究表明，大多数患者的语言和行为评分正常。在采用移植方案的中心中，供体器官短缺导致在等待移植期间约有25%的死亡率，而实际接受器官移植的患者5年生存率与分期重建的患者基本相同。分期重建和移植显著改善了左心发育不全综合征患者的中期预后。预计解决诺伍德手术后早期一期生存率提高的因素将显著改善整体晚期结局。心脏移植后的结局除了受到感染、排斥、移植动脉粥样硬化和淋巴增殖性疾病等晚期并发症的限制外，还受到供体可用性的限制。
