CTICasesSitus Inversus with Polysplenia and Bilateral Left-Sided Bronchial Anatomy
Imaging findings
A patient with a double superior vena cava (left and right SVCs), where the hemiazygos vein drains into the left SVC. The patient has normal levocardia but abdominal imaging reveals situs inversus with polysplenia. Bronchial anatomy shows bilateral left-sided bronchi (bilateral hiparterial bronchi), with a small residual right middle lobe. The patient is relatively asymptomatic but has pulmonary hypertension.
Key takeaways
Situs inversus with polysplenia (a form of heterotaxy syndrome) is associated with a spectrum of complex congenital anomalies, including vascular (e.g., double SVC, hemiazygos continuation), visceral (e.g., situs inversus, multiple spleens), and bronchial (e.g., bilateral left-sided bronchial anatomy) abnormalities. Despite potentially being asymptomatic for many years, these patients have a high incidence of congenital heart disease and require comprehensive cardiac evaluation.
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