Systemic venous anomalies in a child with a vein of Galen.

We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.

An Unusual Presentation of Double-Outlet Right Atrium.

We describe an unusual example of double-outlet right atrium with separate atrioventricular junctions. The straddling and overriding tricuspid valve had two orifices, and the mitral valve was morphologically normal. An appropriate understanding of the morphology of the atrioventricular junctions, the valves, and the subvalvar apparatus, along with the location of the atrioventricular conduction axis, allowed for successful biventricular repair.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

Anomalous systemic venous connection to left atrium in a heart with usual atrial arrangement.

Anomalous systemic venous connection to left atrium is rare anomaly. Previously published cases described this anatomy in patients with left isomerism. Depending on the size of the atrial septal defect, patients usually present with varying degrees of cyanosis and right heart hypoplasia. Here, we report a case of anomalous systemic venous connection to left atrium in a newborn with the usual atrial arrangement.

Device Closure of Superior Sinus Venosus Atrial Septal Defects: A single centre experience.

Sinus venosus atrial septal defects present a wide variety of anatomical features and are frequently associated with partial anomalous pulmonary venous drainage of one or more right pulmonary veins. Surgical correction used to be the standard treatment. In recent times, transcatheter correction of superior sinus venosus atrial septal defects has come into vogue. The transcatheter closure of these defects with covered stents at a tertiary care centre in Oman between 2018 and 2023 is reported.

The Sinus Venosus Veno-Venous Bridge: Not a septal defect.

This review provides an update on the morphology of the sinus venosus defect. It was earlier believed that a 'common wall' separated the right pulmonary veins from the superior caval vein. In the sinus venosus defects, this wall was absent. Current evidence shows that the superior rim of the oval fossa, rather than forming a second septum or representing a common wall, is an infolding between the walls of the caval veins and the right pulmonary veins. The sinus venosus defect is caused by the anomalous connection of one or more pulmonary veins to a systemic vein. However, the pulmonary vein(s) retain their left atrial connections, leading to a veno-venous bridge that allows interatrial shunting outside the oval fossa. True atrial septal defects are located within the oval fossa or in the anteo-inferior buttress, while sinus venosus defects, ostium defects and coronary sinus defects are morphologically distinct from them.

Pulmonary Hemorrhage After Arterial Switch Operation.

Major aortopulmonary collateral arteries can influence the postoperative course of arterial switch operation, with heart failure being the common clinical presentation. A relatively rare presentation of an aortopulmonary collateral artery in the form of persistent postoperative pulmonary hemorrhage was encountered after an uneventful arterial switch operation in a neonate with transposition of the great arteries, intact interventricular septum, and situs inversus totalis. The aortopulmonary collateral artery was coil embolized with a successful outcome.