Systematic review of inferior vena cava atresia

Multimodal imaging assessment for feasibility of endovascular reconstruction in cases of inferior vena cava atresia

“…Surgical interventions, including open venous reconstruction or EVR, may be warranted in those patients with severe symptoms refractory to other treatments. 1,4 With supportive imaging from high-quality CT at the time of initial evaluation and invasive venography at the time of intervention as well as the advancement of endovascular reentry devices, endovascular reconstruction has become possible. 7 Approximately 13% of patients with IVC atresia will undergo EVR.…”

“…7 Approximately 13% of patients with IVC atresia will undergo EVR. 1 Despite the potential for significant symptom improvement and the suggested safety outcomes in published literature, careful case selection is needed due to the complexity and heterogeneity of the anatomy in cases of IVC atresia. 1,6 EVR is thought to be feasible in any case in which a wire can be safely passed across the atretic segment of the IVC.…”

“…IVC atresia, on the other hand, is due to a partial failure of proper development of any of the primordial veins or a secondary process such as IVC thrombosis that leads to a significant reduction in the IVC lumen. 1 In these conditions, deoxygenated blood returns to the right atrium through a robust collateral venous system. 2 IVC atresia is a rare condition with a prevalence of 0.0005%-1% of the population.…”

“…2 IVC atresia is a rare condition with a prevalence of 0.0005%-1% of the population. 1,2 It can be asymptomatic due to collateral flow and discovered incidentally in approximately 25%-33% of cases. 1,3 However, patients may present with a myriad of symptoms such as painful engorgement of superficial collateral veins, lower extremity swelling, venous thrombosis (VT) at a young age, non-healing venous ulcers, and rarely renal dysfunction.…”

“…1,2 It can be asymptomatic due to collateral flow and discovered incidentally in approximately 25%-33% of cases. 1,3 However, patients may present with a myriad of symptoms such as painful engorgement of superficial collateral veins, lower extremity swelling, venous thrombosis (VT) at a young age, non-healing venous ulcers, and rarely renal dysfunction. Premature development of lower extremity VT is the most common initial presentation of this condition.…”