Noyes B E, Kurland G, Orenstein D M, Fricker F J, Armitage J M
Children's Hospital of Pittsburgh, Pennsylvania.
J Pediatr. 1994 Feb;124(2):261-8. doi: 10.1016/s0022-3476(94)70315-9.
Heart-lung transplantation and lung transplantation have become accepted techniques in adult patients with end-stage cardiopulmonary disease. We report here our experience between July 1985 and March 1993 with 34 children (< 20 years) who underwent heart-lung (n = 18) or lung transplantation (n = 17). Indications for transplantation included cystic fibrosis (n = 9), congenital heart disease with Eisenmenger complex (n = 9), primary pulmonary hypertension (n = 8), pulmonary arteriovenous malformations (n = 2), desquamative interstitial pneumonia (n = 2), Proteus syndrome with multicystic pulmonary disease (n = 1), graft-versus-host disease (n = 1), rheumatoid lung disease (n = 1), and bronchiolitis obliterans and emphysema (n = 1). Twenty-six patients (76%) have survived from 1 to 88 months after transplantation; most patients have returned to an active lifestyle. Of the eight deaths, four were due to infections, two to multiorgan failure, 1 to posttransplant lymphoproliferative disease, and one to donor organ failure. Four of the patients who died had cystic fibrosis. Despite considerable morbidity related to infection, rejection, and function of the heart-lung and lung allograft in some patients, our results with this potentially lifesaving procedure in the pediatric population have been encouraging.
心肺移植和肺移植已成为终末期心肺疾病成年患者可接受的治疗技术。我们在此报告1985年7月至1993年3月期间34例儿童(<20岁)接受心肺移植(n = 18)或肺移植(n = 17)的经验。移植适应证包括囊性纤维化(n = 9)、艾森曼格综合征先天性心脏病(n = 9)、原发性肺动脉高压(n = 8)、肺动静脉畸形(n = 2)、脱屑性间质性肺炎(n = 2)、伴有多囊肺疾病的变形综合征(n = 1)、移植物抗宿主病(n = 1)、类风湿性肺病(n = 1)以及闭塞性细支气管炎和肺气肿(n = 1)。26例患者(76%)在移植后1至88个月存活;大多数患者已恢复积极的生活方式。8例死亡患者中,4例死于感染,2例死于多器官功能衰竭,1例死于移植后淋巴细胞增生性疾病,1例死于供体器官功能衰竭。4例死亡患者患有囊性纤维化。尽管一些患者存在与感染、排斥反应以及心肺和肺移植器官功能相关的相当大的发病率,但我们在儿科人群中采用这种可能挽救生命的手术所取得的结果令人鼓舞。