Bilateral sequential lung transplantation in Kartagener syndrome

Abstract

Video clip is available online. Kartagener syndrome (KS) is a rare congenital disease characterized by a triad of sinusitis, bronchiectasis, and situs inversus. Bronchiectasis can progress to end-stage lung failure, and lung transplantation (LTx) might be the only effective treatment. LTx in patients with KS has been technically challenging; however, available reported data are scarce. We have described 2 cases of bilateral sequential LTx in patients with KS and highlighted the pitfalls encountered. The institutional review board of the University of Hong Kong approved the study protocol, and the included patients provided written informed consent. CASE REPORT Patient 1Our first patient was 40-year-old nonsmoking woman with KS and advanced bronchiectasis requiring repeated hospital admissions for infective exacerbation. She had been listed for LTx in 2013 and had undergone bilateral sequential LTx in 2015. The donor and recipient characteristics are listed in Table 1. During the recipient procedure, a clamshell incision was used, and chest entry was performed through the fourth intercostal space. We inserted a Medtronic EOPA 18F arterial cannula and Medtronic Carmeda venous cannula (Medtronic, Dublin, Ireland) into the ascending aorta and right atrium, respectively, and venoarterial extracorporeal membrane oxygenation (ECMO) support using the Cardiohelp system (Maquet, Rastatt, Germany) was begun. The recipient's morphologically right (anatomically left) lung was explanted. The right main bronchus was anastomosed, followed by left atrial anastomosis. The donor pulmonary artery (PA) had to be transposed in a posterosuperior direction to anastomose it to the superiorly located recipient PA. On the left side, the donor PA was displaced anteriorly, circumventing the left main bronchus (Figure 1, A). The remainder of the left lung implant was performed in accordance with standard practice. The duration of ECMO support was 5 hours, 44 minutes. Because of the significant size mismatch, nonanatomic lung volume reduction over the right middle lobe

Unveiling the complexities of lung transplantation in situs inversus

“…Our case report adds to the existing literature on lung transplantation in situs inversus by providing valuable insight into the surgical management and outcomes of this rare scenario. Prior reports specified the necessity of anastomosing the donor pulmonary artery to the side of the recipient pulmonary artery [6], or performing endto-end anastomosis by swapping the donor pulmonary artery between the epibronchial and prebronchial locations [3]. A previous study strongly stated the importance of preserving a longer donor bronchus to mitigate any tension [1,7].…”

Bridge to second double lung transplant with an extracorporeal carbon dioxide removal system in situs inversus patient

“…Lung transplantation in patients with Kartagener's syndrome and situs inversus challenges include dextrocardia with discrepancies between donor and recipient vessels and airways 5–8 . In addition, the chest cavity mismatch between the donor right lung between and the recipient's dextrocardia can result in right lower lobe atelectasis and kinks of the pulmonary artery.…”