Lung transplantation for Kartagener syndrome: technical aspects and morphological adaptation of the transplanted lungs

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Lung transplantation for Kartagener syndrome: technical aspects and morphological adaptation of the transplanted lungs Haruchika Yamamoto1 · Seiichiro Sugimoto1   · Kentaroh Miyoshi1 · Shinji Otani1 · Masaomi Yamane1 · Shinichi Toyooka1 Received: 5 August 2020 / Accepted: 29 September 2020 © The Japanese Association for Thoracic Surgery 2020

Abstract While technical considerations in lung transplantation for Kartagener syndrome have been discussed, little information is available about the postoperative morphological changes of the grafted lungs. Herein, we discuss both the technical aspects and postoperative morphological adaptation of the grafted lungs in a case of Kartagener syndrome. A 46-year-old male patient with Kartagener syndrome underwent bilateral cadaveric lung transplantation. The right arterial anastomosis for transplantation of the size-matched grafts required technical elaboration. After the transplantation, we found a free space in the cardiac notch of the left lung and partial collapse of the lower lobe of the right lung due to dextrocardia. Follow-up computed tomography performed on day 42 after the transplantation demonstrated resolution of the atelectasis and morphological adaptation of the grafts into the recipient’s chest cavity with dextrocardia. Considering such early morphological adaptation of size-matched grafts, lobar reduction could be avoided in lung transplantation for Kartagener syndrome. Keywords  Kartagener syndrome · Lung transplantation · Morphology · Technique · Surgery Abbreviations CT Computed tomography CPB Cardiopulmonary bypass KS Kartagener syndrome LT Lung transplantation PA Pulmonary artery

Introduction Kartagener syndrome (KS) is a rare congenital disorder associated with primary ciliary dyskinesia that is clinically characterized by the triad of situs inversus totalis, bronchiectasis, and chronic sinusitis. Lung transplantation (LT) has been demonstrated as a feasible therapeutic option for patients with KS who present with progressive bronchiectasis and Presented at the 37th annual meeting and scientific sessions of the International Society for Heart and Lung Transplantation, San Diego, CA, USA, April 2017. * Seiichiro Sugimoto sugimo‑[email protected]‑u.ac.jp 1



Department of General Thoracic Surgery and Organ Transplant Center, Okayama University Hospital, 2‑5‑1 Shikata‑cho, Kita‑ku, Okayama 700‑8558, Japan

chronic respiratory failure, with similar reported long-term outcomes to those of LT for other diseases [1–6]. In LT for KS, an elaborate surgical technique is required to adjust for the orientation mismatch of the hilar structures between the recipient and the donor, especially for pulmonary arterial anastomosis, as applied in inverted LT [1–7]. Moreover, the normal anatomical lungs from the donor are morphologically necessitated to fit into the recipient’s chest cavity in the presence of situs inversus with dextrocardia. Morphological mismatch between the donor normal lung and the recipient’s dextrocardia might limit full expansion of the r