Vena Cava Atresia and Deep Vein Thrombosis: A Case Report and Systematic Review

Abstract Congenital anomalies of the vena cava often go unnoticed, and their incidental finding is frequent in imaging tests performed for another cause in asymptomatic patients. However, an association with the development of thromboembolic disease has been described, mainly in young patients. We report the case of a young woman with deep vein thrombosis associated with vena cava atresia. We conducted a search of the PubMed/Medline database. The search identified 17 articles, of which 5 were selected for extraction and data analysis. Twelve studies were excluded because they failed to match the main criteria. We identified six new cases of deep vein thrombosis associated with vena cava abnormalities, with a mean age of 42.5 years; 83.3% were male. Regarding clinical manifestations, all patients presented as deep vein thrombosis, one case of recurrence, and another case associated with the kidney and inferior vena cava abnormalities with leg thrombosis syndrome. Only one patient had a target triggering factor (cholecystectomy postoperative). The thrombophilia study was negative in all cases and none of the patients died. Treatment included enoxaparin and vitamin K antagonists. This is the first study to report on a systematic review of vena cava atresia associated with deep vein thrombosis in Spain. It shows that in this region, the disease affects young population—even in the absence of risk factors—and is linked to a low mortality. The most frequent presentation form was deep vein thrombosis. Therefore, congenital abnormalities of the vena cava should be suspected in young patients with thromboembolic disease, due to their implications regarding to the duration of anticoagulant treatment, as well as their possible association with other prothrombotic factors.