Management of Sinus Venosus Defects

Sinus venosus defects are not atrial septal defects, but are intra-atrial communications outside of the boundaries of the atrial septum. The superior type is located above and separate from the fossal ovalis, usually adjacent to the superior vena cava and the right upper pulmonary vein. The inferior type is located near the orifice of the inferior vena cava and the right lower pulmonary vein. The goal of surgical repair is closure of the defect with unobstructed drainage of the pulmonary veins to the left atrium and of the vena cava to the right atrium. Numerous techniques have been described, particularly for the repair of the superior vena cava type of defect. Mortality and morbidity should be minimal. The risk of either vena cava or pulmonary vein obstruction is low. Sinus node dysfunction can occur postoperatively, particularly when an incision has been made across the superior vena cava/right atrial junction. There is little long-term data on the functional outcomes following repair of these defects.