Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes

Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic medical record was systematically reviewed for thrombotic risk factors in patients who presented with lower-extremity DVT ( n = 409) at a single centre between 1996 and 2017. Patients with IVC atresia were identified based on imaging and chart review. Differences in demographics and thrombotic risk factors between patients with and without IVC atresia were statistically assessed. Extent and chronicity of DVT on imaging, clinical presentation, treatment, and outcomes were evaluated for all patients with IVC atresia. Results 4.2% of DVT patients (17/409) were found to have IVC atresia; mean age at diagnosis was 25.5 ± 9.4 years. The rate of heritable thrombophilia was significantly higher in patients with IVC atresia compared to patients without IVC atresia (52.9% vs. 17.9%, p