Anomalous drainage of left superior venacava into left atrium

The anomalies of drainage of systemic venous communications to the heart are many. But only few cause significant hemodynamic alteration and thus, complications. When they do, they need to be surgically corrected. We report an unusual case of a patient who was found to have left pulmonary veins draining into the left superior venacava (LSVC), which in turn was opening into the roof of the left atrium that had unroofed coronary sinus morphology. Innominate vein was absent. LSVC was divided cranial to where the pulmonary veins were draining into it. Lower end was suture closed. Proximal part of the LSVC was anastamosed to left pulmonary artery.

Bicuspid aortic valve associated aortopathy: a genetic disease

As the most common congenital heart defect, understanding the etiology and progression of aortopathy in bicuspid aortic valve (BAV) is imperative to management of patients with BAV. A reasonable hypothesis, based on the strength of evidence for both genetic and hemodynamic causes of BAV-associated thoracic aortic disease (TAD), is that BAV is caused by genetic variant(s) that also predispose to TAD by a common mechanism; presumably by cell-signaling resulting in an embryologic defect that causes BAV and a postnatal risk of TAD that is accentuated by hemodynamic stress of abnormal flow through the BAV valve. Clinical heterogeneity seen in BAVassociated TAD is likely due to individual genetic variation and the severity of hemodynamic alteration.