Autor(es): Nunes Figueiredo, Catarina ; Santos Silva, João ; Maciel, João ; Semedo, Luísa ; Calvinho, Paulo
Data: 2024
Origem: Portuguese Journal of Cardiac Thoracic and Vascular Surgery
Assunto(s): Kartagener Syndrome; Primary ciliary dyskinesia; situs inversus; double-lung transplant
Kartagener syndrome (KS) is a rare congenital disorder, characterized by sinusitis, bronchiectasis and situs inversus. Lung transplantation is an effective treatment for end-stage lung failure, but dextrocardia and differences between hilar structures and pulmonary lobes require adjustments to conventional surgical technique. We present a case of a double-lung transplant without extracorporeal oxygenation in a 48-year-old male patient with KS. Through a Clamshell incision, right-left rotation was identified. To provide an end-to-end arterial and bronchial anastomosis, longer donor PA’s and right main bronchus were preserved. Wedge resection of right inferior lobe was unnecessary and there was no left residual cardiac chamber. Patient was discharged 32 days after surgery without complications. Despite being anatomically challenging, lung transplant was done successfully without the need for plastic maneuvers or extracorporeal circulation. This reinforces the idea that it should be an option in end-stage lung disease.
