Technical Challenges in Lung Transplantation of Kartagener Syndrome Recipients: A Unique Team Experience With 12 Patients

• Published in: Transplantation proceedings Vol. 52; no. 5; pp. 1384 - 1387
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-06-2020
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2020.02.031

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.
•Kartagener syndrome is a rare congenital disorder related to bronchiectasis, chronic sinusitis, and situs inversus, predisposing the patient to recurrent respiratory infections that can evolve to end-stage lung disease.•Special perioperative care is needed when lung transplantation is indicated in these groups of patients.•Operative concerns include the arteriotomy and bronchotomy during the back-table preparation of the graft, as well as concern about the length of the arterial and bronchial anastomosis.•We found a higher incidence of bronchial complications is this group, which was not previously reported in the literature.