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

<jats:p> 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. </jats:p>

Intramedullary spinal cord abscess associated with right-to-left shunt via right superior vena cava draining into left atrium: A case report

“…[ 5 , 8 ] However, in adult patients, even after complete asymptomatic normal growth and development, this systemic venous anomaly can cause hypoxemia, [ 8 ] myocardial infarction, [ 17 ] stroke or transient ischemic attack, [ 7 , 15 , 17 ] and brain abscess, [ 6 , 12 , 13 , 15 ] with the need for surgical correction of this systemic venous anomaly varying among individual cases. [ 5 ]…”

“…[8] Since its first identification in 1956, [16] there have been approximately 60 reported cases of RSVC-LA, and most patients are diagnosed in the neonatal period or childhood because the shunt leads to hypoxemia and cyanosis. [5,8] However, in adult patients, even after complete asymptomatic normal growth and development, this systemic venous anomaly can cause hypoxemia, [8] myocardial infarction, [17] stroke or transient ischemic attack, [7,15,17] and brain abscess, [6,12,13,15] with the need for surgical correction of this systemic venous anomaly varying among individual cases. [5]…”

“…[2][3][4] The right superior vena cava (RSVC) draining into the left atrium (LA) is an uncommon systemic venous anomaly that results in an RL shunt. [5] This RSVC-LA RL shunt is typically identified in the neonatal period or childhood because of hypoxemia; however, hypoxemia, stroke, myocardial infarction, and brain abscess are seen in completely asymptomatic adult patients with normal growth and development. [6][7][8] Here, we describe the first case of ISCA with an RSVC-LA RL shunt.…”

Anomalous Connection of Superior Vena Cava to Left Atrium—A Rare Congenital Cardiac Anomaly and Its Implications

“…The anomalies of systemic venous return are a heterogeneous group of congenital malformations and can be associated with partial anomalous pulmonary venous drainage, heterotaxy syndrome or may occur as an isolated anomaly. 2,3 The overall incidence of SVC draining into LA is about 0.5% of all cardiac anomalies. 4 After a literature search, a total of 45 case reports of drainage of SVC into LA were found to have been reported so far.…”

“…4 After a literature search, a total of 45 case reports of drainage of SVC into LA were found to have been reported so far. 2 The usual association of SVC to LA drainage includes: atrial or ventricular septal defect; single atrium or single ventricle; transposition of great arteries or tetralogy of Fallot; abnormalities of IVC drainage, patent ductus arteriosus, pulmonary arteriovenous fistulas and coarctation of aorta. 4 Exclusive drainage of SVC to LA without any other cardiac defects is a rare cause of right-to-left connection.…”