Sequential Bilateral Lung Transplant in Situs Inversus Totalis and Kartagener Syndrome

Kartagener syndrome is a rare manifestation of primary ciliary dyskinesia characterized by chronic sinusitis, bronchiectasis, and situs inversus. Progressive bronchiectasis may ultimately lead to end-stage respiratory failure requiring lung transplantation.

Although bilateral lung transplantation is feasible in these patients, situs inversus totalis presents distinctive technical challenges related to mirror-image thoracic anatomy, bronchovascular asymmetry, and potential donor-recipient anatomical mismatch.

The authors present a 40-year-old woman with Kartagener syndrome and situs inversus totalis who underwent bilateral lung transplantation due to advanced bronchiectasis and a progressive decline in respiratory function requiring long-term oxygen therapy.

Technical Considerations

Particular attention should be given to donor-recipient size matching, as dextrocardia modifies the available volume of the right hemithorax and may interfere with expansion of the implanted donor lung.

The airway anatomy is reversed, typically with a long anatomical right main bronchus and a short anatomical left main bronchus with early upper-lobe takeoff. This has implications for both anesthetic management and bronchial implantation.

The pulmonary arterial anatomy represents one of the main technical considerations. Preservation of an adequate donor left atrial cuff is advisable to facilitate vascular implantation.

Despite the anatomical differences, extensive modification of the standard lung transplantation technique is not invariably required. Published experience ranges from procedures requiring specific bronchovascular adaptations to successful transplantation using essentially standard implantation techniques adapted to the reversed anatomy.

Conclusion

Lung transplantation in situs inversus totalis is technically demanding but feasible. Detailed preoperative anatomical evaluation, meticulous donor-recipient size matching, preservation of vascular length, and anticipation of bronchial and pulmonary arterial mismatch are fundamental. A thorough understanding of mirror-image hilar anatomy allows established transplantation principles to be adapted safely to this uncommon anatomical configuration.

References

1. Gauthier JM, Takahashi T, Bierhals AJ, Brody SL, Hachem RR, Witt CA, et al. Technical Considerations for Lung Transplantation in Kartagener’s Syndrome. The Annals of Thoracic Surgery. 2019;107:e337–e339. DOI: 10.1016/j.athoracsur.2018.08.095.
2. Kurihara C, Kaiho T, Bharat A. Unveiling the Complexities of Lung Transplantation in Situs Inversus. Surgical Case Reports. 2024;10:24. DOI: 10.1186/s40792-024-01812-1.
3. Marro M, Leiva-Juárez MM, D’Ovidio F, Chan J, Van Raemdonck D, Ceulemans LJ, et al. Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study. Transplant International. 2023;36:10819. DOI: 10.3389/ti.2023.10819.

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