CTICasesObstructed Supracardiac TAPVR and Heterotaxy Syndrome with Asplenia
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Imaging findings
Chest CT angiography in a newborn reveals complex congenital anomalies: a large atrioventricular canal defect, pulmonary atresia with a large patent ductus arteriosus, and supracardiac total anomalous pulmonary venous return. The pulmonary veins form a common vein that is severely obstructed at the level of a small superior vena cava. Massive dilatation of the azygos vein and the vertebral venous plexus is present, indicating collateral retrograde flow. Additionally, there is bilateral right-sided bronchial anatomy, a common atrium, two inferior vena cava-like structures draining the liver, and asplenia.
Key takeaways
Heterotaxy syndrome with bilateral right-sidedness (asplenia) is typically associated with complex, severe congenital cardiac malformations, including obstructed total anomalous pulmonary venous return and pulmonary atresia. Severe venous obstruction in supracardiac TAPVR triggers massive collateral dilatation of the azygos, hemiazygos, and paravertebral venous systems to bypass the obstructed SVC.
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