Sequential Double-Lung Transplantation in Kartagener Syndrome: A Case Report

Abstract

<h3>Introduction</h3> 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. Main surgical difficulties found in these patients are due to dextrocardia and differences between left and right hilar structures and pulmonary lobes, which may cause the need to adapt the usual surgical technique. Only few technical reports were made for lung transplantation in situs inversus. <h3>Case Report</h3> We present a 48-year-old male patient with KS. He was 1,69m and had a TLC of 7,32L. When first evaluated for transplantation in 2019, he had a FEV1 and DLCO of 32% (1.1L) and 38% (3.7L), respectively, with a need of supplemental O2 (2L-rest; 4L-effort). He remained clinical and functionally stable for 2 years and was transplanted in 2021 (donor: 1,75cm, TLC 6,9L). Patient ventilation was performed with left sided double lumen tube that made selective ventilation quite difficult, therefore, an endobronchial blocker was used. A Clamshell incision was made with identification of left-right structure rotation. Without extracorporeal circulation, sequential double-lung transplantation was performed. Despite the evident anatomical changes, donor and recipient ipsilateral lungs had a good morphometric matching. Different anatomic relationships were taken into consideration. Since left recipient hilus had a right anatomy and right recipient hilus had a left anatomy, to provide an end-to-end arterial and bronchial anastomosis, we had to preserve a longer donor PA in both sides, and a longer donor right main bronchus. No technical alterations were made in the auricular cuff. There was no need to perform wedge resection of the right inferior lobe and left residual cardiac chamber wasn't verified. Left and right lung ischemic time were 4h12min and 6h22min, respectively. Patient was extubated 14h post-op and discharged 32 days after surgery with no complications. <h3>Summary</h3> We performed sequential double-lung transplantation in a patient with KS. Due to anatomic relations, longer donor pulmonary arteries and main right bronchus were necessary. There was no need for plastic maneuvers and the surgery was performed in the usual operative time.