Pediatric Cardiology, Queen Silvia Children's Hospital, Sahlgrenska Academy, Gothenburg University, Sweden.
Acta Paediatr. 2011 Nov;100(11):1442-7. doi: 10.1111/j.1651-2227.2011.02377.x. Epub 2011 Jun 23.
To evaluate outcome in the first generation of children with end-stage heart disease to whom heart transplantation was available.
Retrospective review of all 135 Swedish children <18 years old listed for heart transplantation 1989-2009, followed to December 31, 2009, including 74 (55%) with cardiomyopathy and 61 (45%) with congenital heart disease; 34 (25%) were infants (<1 year). Cumulative risk of requiring heart transplantation was 1:17,300 (11 patients who improved were omitted from outcome analysis).
Waiting-list mortality was 31% (44% in infants). Median waiting time in 82 transplanted patients was 57 days (0-585 days). Post-transplant follow-up time was median 5.9 years (0.03-20.1 years), and actuarial survival was 92% at 1 year, 82% at 5 years, 76% at 10 years and 58% at 15 years. Survival after listing was 64% at 1 year, 58% at 5 years, 52% at 10 years and 40% at 15 years. Post-transplant complications included rejections (34%), malignancies (12%), renal failure (8%), coronary artery vasculopathy (6%) and re-transplantation (5%). Among 64 survivors, 84% were free of complications affecting prognosis.
High waiting-list mortality and post-transplant attrition precluded 60% of this pioneer population from reaching adulthood. Functional status in survivors is generally good.
评估可进行心脏移植的第一代终末期心脏病儿童的治疗结果。
回顾性分析了 1989 年至 2009 年瑞典所有 135 名 <18 岁的接受心脏移植的儿童患者，其中包括 74 例（55%）扩张型心肌病和 61 例（45%）先天性心脏病；34 例（25%）为婴儿（<1 岁）。需要心脏移植的累积风险为 1:17300（11 例改善的患者被排除在结果分析之外）。
等待名单上的死亡率为 31%（婴儿为 44%）。82 例移植患者的中位等待时间为 57 天（0-585 天）。移植后中位随访时间为 5.9 年（0.03-20.1 年），1 年生存率为 92%，5 年生存率为 82%，10 年生存率为 76%，15 年生存率为 58%。列表后生存率为 1 年 64%，5 年 58%，10 年 52%，15 年 40%。移植后并发症包括排斥反应（34%）、恶性肿瘤（12%）、肾衰竭（8%）、冠状动脉血管病（6%）和再次移植（5%）。在 64 例幸存者中，84%无影响预后的并发症。
高的等待名单死亡率和移植后失访率使该先驱人群的 60%无法达到成年。幸存者的功能状态通常良好。
