Mahle W T, Spray T L, Wernovsky G, Gaynor J W, Clark B J
Divisions of Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Circulation. 2000 Nov 7;102(19 Suppl 3):III136-41. doi: 10.1161/01.cir.102.suppl_3.iii-136.
There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results.
Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival (P:<0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality (P:=0.004 and P:=0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival.
Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.
关于接受过左心发育不全综合征（HLHS）重建手术患者的长期生存率数据有限。我们回顾了本机构15年的经验，以在早期手术结果持续改善的背景下研究生存率。
1984年至1999年期间，840例患者接受了HLHS的I期手术。通过查阅病历和直接与患者联系来确定生存状况。整个队列的1年、2年、5年、10年和15年生存率分别为51%、43%、40%、39%和39%。在接受Fontan手术后出院回家的291例患者中，有14例发生晚期死亡，不过仅有1例患者在5岁以后死亡。5例患者在I期重建后接受了心脏移植。I期手术的后期与生存率显著提高相关（P<0.001）。1995年至1998年接受I期重建的患者3年生存率为66%，而1984年至1988年接受手术的患者为28%。I期手术时年龄>14天以及I期手术时体重<2.5 kg也与较高的死亡率相关（分别为P=0.004和P=0.01）。其他变量，包括解剖亚型、内脏异位以及后续分期手术时的年龄，与生存率无关。
在本研究的15年过程中，接受分期姑息治疗的HLHS患者的早期和中期生存率显著提高。晚期死亡和心脏移植的需求并不常见。
