Inferior vena cava (IVC) atresia is a rare congenital vascular malformation. We describe the case of a 20-year-old woman with IVC atresia who presented with a 3-month history of fatigue, oedema of the lower limbs and episodes of lipothymia. Transthoracic echocardiography and cardiac catheterization were performed, revealing interruption of the IVC with circulation through the azygos and hemiazygos system. An abdominal and pelvic computerized tomography (CT) scan confirmed the findings, demonstrating the absence of the IVC below the renal veins. Blood tests did not reveal any relevant results. These findings are consistent with the diagnosis of IVC atresia, a rare condition with no standard treatment. As a surgical approach was not possible, pharmacological measures were implemented for primary prevention of possible thrombotic events.
Learning points: Inferior vena cava atresia is a rare vascular malformation.The clinical presentation is non-specific: most diagnoses are made after a thrombotic event or as an incidental finding on imaging.Consensus on definitive treatment is lacking, but the increased thrombotic risk warrants primary prevention of thrombosis.
Keywords: Inferior vena cava; collateral circulation; vascular malformations.
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