Persistent Levoatriocardinal Vein in Association with Complete Duplication of SVC and IVC

Persistent levoatriocardinal vein (LACV) is a rare pulmonary venous anomaly characterized by persistence of embryonic pulmonary to systemic communication. It is commonly associated with obstructive lesions of the left heart, such as hypoplastic left heart syndrome and mitral atresia. Complete duplication of the superior vena cava (SVC) and inferior vena cava (IVC) is rare, and occurs due to aberrations in the multistep development of the cardinal venous system. Coexistence of double SVC and IVC has been reported in the literature; however, their occurrence with persistent levoatriocardinal vein is hitherto unreported. The current case describes a rare coexistence of these complex pulmonary and systemic venous anomalies: persistent levoatriocardinal vein in a child with double SVC and IVC. The embryological development and possible aberrations leading to these anomalies are also detailed.