de Castro Caio César Bianchi, Dos Reis Flávio Pola, de Carvalho Guilherme Vieira Soares, Fernandes Lucas Matos, Abdalla Luis Gustavo, Samano Marcos Naoyuki, Júnior José Eduardo Afonso, Pêgo-Fernandes Paulo Manoel
Lung Transplantation Division, Thoracic Surgery Department, Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Lung Transplantation Division, Thoracic Surgery Department, Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; Lung Transplantation Department, Hospital Israelita Albert Einstein, São Paulo, Brazil.
Transplant Proc. 2020 Jun;52(5):1384-1387. doi: 10.1016/j.transproceed.2020.02.031. Epub 2020 Apr 14.
Kartagener syndrome (KS) is a rare congenital disorder related to bronchiectasis, chronic sinusitis, and situs inversus, predisposing patients to recurrent respiratory infections that can evolve to end-stage lung disease; lung transplantation (LTx) is one of the therapeutic options. This study highlights some concerns in this group of patients, mainly related to the difficulty of performing the transplantation in recipients with suppurative disease and situs inversus. We conducted a retrospective analysis of all KS patients who underwent LTx at 2 national reference centers by the same LTx team. During 29 years of analysis, we performed 12 cases of bilateral sequential LTx in KS patients, representing 2.4% of all Ltx that we performed. Special perioperative care is needed, including vascular access sites and lung isolation techniques; operative concerns include the arteriotomy and bronchotomy during the back table preparation of the graft and concern about the length of the arterial and bronchial anastomosis. We found a higher incidence of bronchial complications is this group that had not been previously reported. Bilateral sequential orthotopic LTx is feasible in this group of patients, and more studies are needed to understand possible reasons for the apparent higher incidence of bronchial complications.
卡塔格内综合征（KS）是一种罕见的先天性疾病，与支气管扩张、慢性鼻窦炎和内脏转位有关，使患者易反复发生呼吸道感染，进而可能发展为终末期肺病；肺移植（LTx）是治疗选择之一。本研究强调了该组患者的一些问题，主要涉及在患有化脓性疾病和内脏转位的受者中进行移植的困难。我们对同一肺移植团队在2个国家参考中心接受肺移植的所有KS患者进行了回顾性分析。在29年的分析期间，我们对KS患者进行了12例双侧序贯肺移植，占我们所进行的所有肺移植的2.4%。需要特殊的围手术期护理，包括血管接入部位和肺隔离技术；手术问题包括在移植器官后台准备期间的动脉切开术和支气管切开术，以及对动脉和支气管吻合长度的担忧。我们发现该组支气管并发症的发生率较高，此前尚未有报道。双侧序贯原位肺移植在该组患者中是可行的，需要更多研究来了解支气管并发症明显较高发生率的可能原因。
