Sigfússon G, Fricker F J, Bernstein D, Addonizio L J, Baum D, Hsu D T, Chin C, Miller S A, Boyle G J, Miller J, Lawrence K S, Douglas J F, Griffith B P, Reitz B A, Michler R E, Rose E A, Webber S A
Department of Pediatrics, University of Pittsburgh, Pennsylvania, USA.
J Pediatr. 1997 Jun;130(6):862-71. doi: 10.1016/s0022-3476(97)70270-1.
Short-term survival after pediatric heart transplantation is now excellent, but ultimately the efficacy of this procedure will depend on duration and quality of survival. We sought to evaluate the clinical course of long-term survivors of heart transplantation in childhood.
Patients who had undergone heart transplantation at the university hospitals of Stanford, Columbia, and Pittsburgh between 1975 and 1989 and survived longer than 5 years from transplantation were identified and their clinical courses retrospectively reviewed.
Sixty eight children have survived more than 5 years from transplantation, and 60 (88%) are currently alive with a median follow-up of 6.8 years (5 to 17.9 years). Thirteen have survived more than 10 years from transplantation. Renal dysfunction caused by immunosuppressive agents was common, and two patients required late renal transplantation. Lymphoproliferative disease or other neoplasm occurred in 12 patients, but none resulted in death. Coronary artery disease was diagnosed in 13 patients (19%), leading to retransplantation in eight. Death after 5 years was related to acute or chronic rejection in 5 of 8 cases. Two of the deaths were directly related to noncompliance with immunosuppressive medication. All survivors are in New York Heart Association class 1.
Long-term survival with good quality of life can be achieved after heart transplantation in childhood, though complications of immunosuppression remain common. Posttransplantation coronary artery disease is emerging as the main factor limiting long term graft and patient survival.
小儿心脏移植后的短期生存率目前已非常理想,但该手术的最终疗效将取决于生存时间和质量。我们试图评估儿童心脏移植长期存活者的临床病程。
确定1975年至1989年间在斯坦福大学医院、哥伦比亚大学医院和匹兹堡大学医院接受心脏移植且移植后存活超过5年的患者,并对其临床病程进行回顾性分析。
68名儿童移植后存活超过5年,60名(88%)目前仍存活,中位随访时间为6.8年(5至17.9年)。13名患者移植后存活超过10年。免疫抑制剂导致的肾功能障碍很常见,2名患者后期需要进行肾移植。12名患者发生了淋巴增生性疾病或其他肿瘤,但均未导致死亡。13名患者(19%)被诊断出患有冠状动脉疾病,其中8名因此接受了再次移植。8例5年后死亡的病例中,5例与急性或慢性排斥反应有关。2例死亡与未遵守免疫抑制药物治疗直接相关。所有存活者均为纽约心脏协会1级。
儿童心脏移植后可实现长期存活且生活质量良好,尽管免疫抑制的并发症仍然常见。移植后冠状动脉疾病正逐渐成为限制移植物和患者长期存活的主要因素。