Systematic review of inferior vena cava atresia

Objective: Inferior vena cava (IVC) atresia is a rare venous anomaly characterized by absence of the IVC. It has been associated with deep vein thrombosis (DVT) and other congenital anomalies. The aim of the present study is to provide a comprehensive summary of the literature on IVC atresia and discuss the presentation and outcomes of patients with IVC atresia.Methods: A systematic review of the English literature up to April 2020 was performed. The presentations and treatments reported were noted and compared between the two sexes. The IVC atresia cases were further stratified into isolated IVC atresia and IVC atresia associated with other congenital anomalies.Results: A total of 412 abstracts were screened, with 178 reports included. A total of 376 patients were analyzed. Overall, males seem to be more affected than females, with a ratio of almost 2:1 (male, 227 [64.1%]; vs female, 127 [35.8%]). However, females were more likely to have congenital IVC atresia compared with males (46.1% vs 21.3%; P < .001). The mean age at presentation was 27.9 6 18.0 years (range, 0-77 years), with no differences between the sexes. Most patients with IVC atresia presented with DVT (n = 242 of 376; 64.3%), with the iliac veins most often affected (n =159 of 242; 65.7%). No difference was found in the reported proportion of patients presenting with DVT between the two sexes. The symptom presentation was similar, with leg pain and swelling the most common in both sexes. The patients were treated either medically with anticoagulation or surgically (open or endovascular). No mortality was reported with isolated IVC atresia in either treatment group. However, the mortality of patients with IVC atresia associated with other congenital anomalies was 11.7%.Conclusions: IVC atresia is more common in males but seems to have a predilection for females in the setting of other congenital anomalies. Most patients present with leg pain and swelling related to the development of DVT. Open and endovascular surgical interventions to treat IVC atresia have been reported in 18.3% of patients reviewed, with acceptable mid-term results in terms of patency and symptomatic relief.