A Review of the Surgical Management of Anomalous Connection of the Right Superior Caval Vein to the Morphologically Left Atrium and Biatrial Drainage of Right Superior Caval Vein

Abstract

The present perspective is a synthesis of published investigations in the setting of anomalous connection of the right superior caval vein to the morphologically left atrium or biatrial drainage of the right caval vein. We identified 57 suitable cases from 97 investigations, reviewing the clinical presentation, diagnostic modalities utilized, surgical techniques used, and their outcomes. Clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, radionuclide perfusion scan, magnetic resonance imaging, and angiocardiography provided the diagnostic information and were used to define the disease entities before surgery. We have also addressed several issues concerning the influence of the so-called heterotaxy: the establishment of the diagnosis, the variation in clinical presentation, and subsequent management. For the overall group of patients undergoing either surgical intervention or transcatheter treatment with an Amplatzer vascular plug, the operative mortality remains high at 9.5%. We submit that an increased appreciation of these disease entities will contribute to improved future surgical management.

Keywords: anomalous systemic venous drainage; biatrial drainage of right superior vena cava; congenital heart disease; cyanosis; left superior vena cava; partially anomalous pulmonary venous connection; polycythemia; pulmonary vein stenosis; right superior vena cava; sinus venosus atrial septal defect; totally anomalous systemic venous connection.