Interrupted Inferior Vena Cava Associated with Ebstein’s Anomaly: A Rare Case Report

Abstract

Background: The complete absence hepatic portion of the inferior vena cava (IVC) is known as “interruption” of the IVC. Congenital interruption of the IVC can rarely occur in patients with a normal visceroatrial situs. It is an uncommon vascular anomaly caused by failure in embryonic development. It can occur in isolation or in association with congenital heart defects. Case Presentation: We report a 53-year-old man presenting with progressive dyspnea. Clinical examination revealed signs of right-sided heart failure. Electrocardiography demonstrated atrial fibrillation with a ventricular rate of 100 bpm. Transthoracic echocardiography showed type I Ebstein’s anomaly, severe tricuspid regurgitation, hypoplastic pulmonary artery with severe valvular stenosis, and IVC interruption. CT angiography confirmed agenesis of the middle and lower IVC segments with collateral drainage via the azygos and hemiazygos systems. The patient underwent successful surgical management consisting of bioprosthetic tricuspid valve replacement, pulmonary artery patch augmentation, and atrial septal defect closure. Conclusion: This rare association of interrupted IVC and Ebstein’s anomaly highlights the importance of multimodality imaging in identifying complex cardiovascular malformations and guiding surgical planning