Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

Photographs of a pathologic specimen from the archive of Pittsburgh Children's Hospital (A) and a specimen from the collection at the Hospital for Sick Children (B) demonstrating an inferior sinus venosus defect, a venoatrial communication outside the confines of an intact oval fossa. In A, the arrows point to the outside of the pulmonary veins. In B, the arrows for the inferior pulmonary vein and the defect point to the same hole. This hole exists because the inferior pulmonary vein is connected to the inferior caval vein while retaining its connection to the left atrium. RS pulm. vein, Right superior pulmonary vein; SCV, superior caval vein; RI pulm. veins, right inferior pulmonary veins; SV, sinus venosus; ICV, inferior caval vein; PV, pulmonary vein; LSCV, left superior caval vein.

The image demonstrates the echocardiographic subcostal right anterior oblique view of the inferior sinus venosus defect. The white arrow points at the most inferior margin of the intact atrial septum and marks the roof of the venoatrial communication. The asterisk is placed within the right inferior pulmonary vein and demonstrates the anomalous connection to the right atrium. LA, Left atrium; RVOT, right ventricular outflow tract; ICV, inferior caval vein; RA, right atrium; RV, right ventricle.