Division of Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine in St. Louis, St. Louis, Missouri.
Division of Diagnostic Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine in St. Louis, St. Louis, Missouri.
Ann Thorac Surg. 2019 May;107(5):e337-e339. doi: 10.1016/j.athoracsur.2018.08.095. Epub 2018 Oct 26.
Kartagener's syndrome is a rare genetic disorder of ciliated epithelial cells associated with recurrent respiratory tract infections, bronchiectasis, and situs inversus. In some patients, the accumulation of airway secretions and recurrent infections lead to end-stage lung disease, for which lung transplantation is the only effective treatment. Anatomical variations, such as dextrocardia and pulmonary situs inversus, make the procedure challenging, yet feasible with certain technical modifications and careful preparation of donor lungs. We report a case of bilateral lung transplantation without the use of cardiopulmonary bypass in a patient with Kartagener's syndrome while describing important technical details of the operation.
卡塔格内氏综合征是一种罕见的纤毛上皮细胞遗传疾病，常伴有复发性呼吸道感染、支气管扩张和内脏转位。在一些患者中，气道分泌物的积聚和反复感染导致终末期肺病，肺移植是唯一有效的治疗方法。解剖变异，如右位心和肺脏转位，使手术具有挑战性，但通过某些技术修改和供肺的精心准备，仍然可行。我们报告了一例卡塔格内氏综合征患者在不使用体外循环的情况下进行双侧肺移植的病例，并描述了手术的重要技术细节。
