Bilateral Lung Transplantation in Kartagener's Syndrome and Situs Inversus.

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.