CTICasesCongenital Soup (Situs Ambiguus / Left Isomerism / Malrotation)
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Imaging findings
Chest radiograph shows dextrocardia. CT reveals dextrocardia with an inverted left SVC, an enlarged azygos/hemiazygos vein, and the aorta on the right. There is left isomerism of the lungs with two hype-arterial bronchi and a tracheal bronchus on the left. Abdominal imaging shows polysplenia on the left (consistent with left-sidedness), a left IVC, and gut malrotation with small bowel on the right and colon on the left. Despite these extensive anomalies, there are no specific shunts or cardiac malformations other than dextrocardia and coronary artery disease/dysrhythmia (with a pacemaker).
Key takeaways
This case represents a complex constellation of congenital anomalies, termed "congenital soup," indicative of situs ambiguus with features of left isomerism (polysplenia, left SVC, right-sided aorta, left IVC). These patients often have multiple congenital anomalies, including airway abnormalities (e.g., tracheal bronchus) and gut malrotation. While cardiac malformations are common, this patient surprisingly had pure dextrocardia without shunts, presenting primarily with dysrhythmias and requiring a pacemaker.
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