Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes
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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.
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.
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 < 0.0001). There were bilateral DVT in 70.6% of IVC atresia patients; DVT was chronic in 41.2% and acute on chronic in 58.8%. Pre-intervention Villalta scores were 13.9 ± 9.8 in the left limb and 8.5 ± 7.0 in the right limb. DVT in IVC atresia patients was typically treated with catheter-directed thrombolysis followed by stent placement, achieving complete or partial symptom resolution in 78.6% of cases.
Thrombotic risk factors such as heritable thrombophilia are associated with IVC atresia. IVC atresia patients can experience high burdens of lower-extremity thrombotic disease at a young age which benefit from endovascular treatment.
Level of Evidence
KeywordsInferior vena cava atresia Deep vein thrombosis Endovascular intervention Thrombophilia
This study was funded by the Stanford Medical Scholars Grant, and the Stanford Department of Radiology. The funders had no role in study design, data collection, analysis, or interpretation, decision to publish, or preparation of the manuscript.
Compliance within Ethical Standards
Conflict of interest
L.V.H. is a paid consultant for Cook, Inc. (Bloomington, Indiana) and W.L. Gore & Associates (Flagstaff, Arizona); is a shareholder in Cook, Inc.; owns equity in Confluent Medical (Scottsdale, Arizona), Boston Scientific (Marlborough, Massachusetts), and Medtronic (Minneapolis, Minnesota); and is the founder of, on the board of directors for, and owns equity in Grand Rounds (San Francisco, California). None of the other authors have identified a conflict of interest.
This study is approved by the Stanford Institutional Review Board (#33192). For this retrospective study, formal consent is not required.
For this type of study informed consent is not required.
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For this type of study consent for publication is not required.
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