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Case Reports
. 2023 Mar 5;15(3):e35785.
doi: 10.7759/cureus.35785. eCollection 2023 Mar.

Bilateral Lung Transplantation in Kartagener's Syndrome and Situs Inversus

Muhammad Asad Faruqi  1 , Suresh Keshavamurthy  2 , Karl D Hillenbrand  3 , Michael Anstead  4 , Sravanthi Nandavaram  5
Affiliations
Case Reports

Bilateral Lung Transplantation in Kartagener's Syndrome and Situs Inversus

Muhammad Asad Faruqi et al. Cureus. .

Abstract

Kartagener's syndrome (KS) is a genetic disorder and a subgroup of primary ciliary dyskinesia characterized by situs inversus, chronic sinusitis and bronchiectasis. Patients with KS can develop severe bronchiectasis with end-stage lung disease due to recurrent pulmonary infections. Lung transplantation is a treatment option with good outcomes reported in the literature. Lung transplantation in such patients can be technically challenging given the dextrocardia, bronchial asymmetry and anatomical variation of major vascular structures due to situs inversus. We present a case of a 45-year-old male with KS complicated by recurrent infections and chronic respiratory failure, who successfully underwent a bilateral sequential lung transplant (BSLTx). Because of repeated infections and severe bronchiectasis, the patient's quality of life was impaired, and he was oxygen dependent. As a definitive treatment, successful lung transplantation led to a reversal of hypoxic respiratory failure and the patient's symptoms markedly improved, reinforcing data in the literature to consider lung transplantation in this patient population.

Keywords: bilateral anterolateral thoracotomy; kartagener’s syndrome; primary ciliary dyskinesia; situs inversus; veno-arterial-venous extracorporeal membrane oxygenation.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Chest x-ray showing dextrocardia
Figure 2
Figure 2. CT scan of the chest demonstrating severe bronchiectasis.
Figure 3
Figure 3. CT scan showing dextrocardia and bronchiectasis
Figure 4
Figure 4. 3D reconstruction of airways and vessels shows hyp-arterial bronchus on the right side due to situs inversus.
Figure 5
Figure 5. 3D reconstruction of the airways and vessels shows left-sided shorter ep-arterial bronchus.
Figure 6
Figure 6. Chest x-ray post operatively shows newly implanted lungs.
Figure 7
Figure 7. CT chest prior to hospital discharge
Figure 8
Figure 8. CT chest demonstrating transplanted lungs in the patient with Kartagener's syndrome
Figure 9
Figure 9. 3D reconstruction shows newly transplanted lungs.
Figure 10
Figure 10. Right mainstem bronchus shown. Arrows point to the anastomosis between donor and recipient bronchi.
Figure 11
Figure 11. Left mainstem bronchus shown. Arrows point to the anastomosis between donor and recipient bronchi.
See this image and copyright information in PMC

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