A cyanotic 1-day-old boy was diagnosed with double inlet right ventricle with an incomplete left ventricle, pulmonary atresia and left-sided patent arterial duct. After administration of intravenous prostaglandin E1 for 28 days, he underwent construction of a left-sided modified Blalock-Taussig shunt, following which prostaglandin was discontinued. During subsequent outpatient follow-up, he gradually became desaturated and dyspnoeic, with pulse oximetry reading 63%, and audible stridor. These symptoms were suspected to reflect occlusion of the Blalock-Taussig shunt, pulmonary coarctation, and/or constriction of the airways. Contrast-enhanced 16-row multislice computed tomography was performed, and three-dimensional images were reconstructed (Fig. 1). The cardiovascular image (a) showed mild pulmonary coarctation (white arrow) at the site of the arterial duct, which was no longer visible. The modified Blalock-Taussig shunt (open arrow) was seen. Superimposition of the tracheobronchial image (b) demonstrated severe compression of the trachea by the dilated aorta (arrowhead). Bronchoscopy confirmed constriction of the tracheal lumen with pulsation on its left-anterior aspect.

Figure 1.

The patient first underwent transcatheter implantation of a stent across the pulmonary coarctation at 3 months of age to prevent disconnection of the pulmonary artery, but this occasioned no significant improvement in saturations of oxygen or dyspnoea. Accordingly, aortopexy was performed one week later to relieve tracheal compression. At this operation, it was confirmed that the atrial appendages were arranged in mirror-imaged fashion. Following the procedure, pulse oximetry improved to a saturation of 77%, and stridor was reduced. One month postoperatively, computed tomography was repeated, and three-dimensional reconstruction (Fig. 2) visualized both the stent (blue) at the site of pulmonary coarctation (a), and marked relief of tracheal constriction (arrowhead – b).

Figure 2.