Sequential Double-Lung Transplantation In Kartagener Syndrome: A Case Report
DOI:
https://doi.org/10.48729/pjctvs.465Keywords:
Kartagener Syndrome, Primary ciliary dyskinesia, situs inversus, double-lung transplantAbstract
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.
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References
Abbasi MA, Suleman A, Ahmed N, Zaman H. Kartagener syndrome: a not so rare phenomenon. J Ayub Med Coll Abbottabad. 2014;26(4):598–601.
Knowles MR, Zariwala M, Leigh M. Primary Ciliary Dyskinesia. Vol. 37, Clinics in Chest Medicine. W.B. Saunders; 2016. p. 449–61.
Leigh MW, Pittman JE, Carson JL, Ferkol TW, Dell SD, Davis SD, et al. Clinical and genetic aspects of primary ciliary dyskinesia/kartagener syndrome. Vol. 11, Genetics in Medicine. 2009. p. 473–87.
Gauthier JM, Takahashi T, Bierhals AJ, Brody SL, Hachem RR, Witt CA, et al. Technical Considerations for Lung Transplantation in Kartagener’s Syndrome. Annals of Thoracic Surgery. 2019 May 1;107(5):e337–9.
Mishra M, Kumar N, Jaiswal A, Verma AK, Kant S. Kartagener’s syndrome: A case series. Lung India. 2012 Oct;29(4):366–9.
Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, et al. Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatr Pulmonol. 2016 Feb 1;51(2):115–32.
Hayes D, Reynolds SD, Tumin D. Outcomes of lung transplantation for primary ciliary dyskinesia and Kartagener syndrome. Vol. 35, Journal of Heart and Lung Transplantation. Elsevier USA; 2016. p. 1377–8.
Wang B, Zhang X, Jiang W, Huang J, Chen J, Kreisel D, et al. Double lung transplantation for end-stage Kartagener syndrome: A case report and literature review. Vol. 12, Journal of Thoracic Disease. AME Publishing Company; 2020. p. 1588–94.
Lucius Lee KF, Michael Hsin KY, Alva Sit KY, Cally Ho KL, Timmy Au WK. Bilateral sequential lung transplantation in Kartagener syndrome. JTCVS Tech. 2020 Sep;3:406–8.
Lama Martínez R, Santos Luna F, Salvatierra Velázquez A, Cerezo Madueño F, Algar Algar J, Alvarez Kindelán A. A sequential double lung transplant in Kartagener’s syndrome. Arch Bronconeumol. 2000;36(2):106–8.
Macchiarini P, Chapelier A, Vouhe P, Cerrina J, Le F, Ladurie R, et al. Double lung transplantation in situs inversus with Kartagener’s syndrome. 1994.
Mentzer SJ, Aranki SF, Reilly JJ, DeCamp MM, Hartigan P, O’Donnell W, et al. Single-lung transplantation in situs inversus. Ann Thorac Surg. 1994 Oct;58(4):1176–8.
Graeter T, Schäfers HJ, Wahlers T, Borst HG. Lung transplantation in Kartagener’s syndrome. J Heart Lung Transplant. 1994;13(4):724–6.
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