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artagener syndrome is a rare congenital genetic dis- order that is characterized by the triad situs inversus, bronchiectasis, and sinusitis.1 Patients have defective ciliary motility that leads to reduced or absent mucus clearance in the lungs and susceptibility to chronic respiratory infections. Lung transplantation has been shown to be effective in cases of severe respiratory failure. Only a few cases of lung transplantation in patients with Kartagener syndrome have been reported so far.2 Multidetector row computed tomography (MDCT) represents the principal diagnostic modality for the assessment of broncho- pulmonary and cardiovascular anatomy. Recent developments of MDCT technology, including CT angiography (CTA), enable the acquisition of near-isotropic datasets representing the prerequisite for 2- and 3-dimensional (2D and 3D) postprocessing techniques. We describe the case of a patient with Kartagener syndrome in whom MDCT with 2D and 3D postprocessing allowed a precise visualization of bronchopulmonary and cardiovascular morpho- logic characteristics before and after lung transplantation. Clinical Summary The diagnosis of Kartagener syndrome with situs inversus totalis thoracalis et abdominalis was made in a 59-year-old patient at the age of 19 years. At the age of 13 years, a lower bilobectomy (middle and lower lobes) was performed on the left side because of symptomatic bronchiectasis. In recent years, the patient had global respiratory insufficiency. Bilateral lung transplantation was planned. Preoperative MDCT was performed for surgical treatment planning, and images were postprocessed with multiplanar refor- mations (MPR), surface-shaded display (SSD), and volume ren- dering (VR) technique. The CT showed dextrocardia, a right-sided ascending aorta and aortic arch, and multiple bronchiectasis in the (right) lingula and left upper lobe (Figure 1). Furthermore, the preoperative CT scan revealed the direction and exact length of the main stem bronchi for facilitating surgical planning. Virtual bron- choscopic images using the SSD and VR techniques revealed an irregularly shaped distal trachea and the bronchial stump after left middle and lower lobe resection (Figure 1). Lung transplantation was performed 8 months after the patient had been put on the waiting list. For the sequential bilateral lung transplantation, a clamshell approach (combination of two antero- lateral thoracotomies with a transverse sternotomy) was chosen. This access was used to provide optimal exposure in this patient with situs inversus. Extracorporeal circulation was not required. The left donor lung was first transplanted on the left recipient side and then the right donor lung on the right recipient side. Postop- erative recovery was uneventful. VR combined with CTA images demonstrated a kinking of the right pulmonary artery without hemodynamically relevant stenosis (Figure 2).

journal of thoracic and cardiovascular surgery

Lung transplantation in Kartagener syndrome and situs inversus: Potential of multidetector row computed tomography and three-dimensional postprocessing