Echocardiographic diagnosis and repair of an inferior sinus venosus defect

Abstract

Transesophageal echocardiography in an asymptomatic nine-month-old girl discerned features of an inferior sinus venosus defect, demonstrating left-to-right shunting across a posteroinferior interatrial connection (Figures 1A and 1B) near the inferior vena cava, and a small, separate patent foramen ovale (Figure 1C). The right inferior pulmonary vein entered the left atrium more obliquely than normal, and the right atrium and right ventricle were slightly dilated. The diagnosis was confirmed intraoperatively during subsequent elective pericardial patch repair: the defect was located outside the intact muscular limbus, unrelated to the oval fossa and patent foramen ovale (Figure 2), and was bounded inferiorly by an overriding inferior vena cava. These features, with or without anomalous pulmonary venous drainage, distinguish this rare congenital defect from posterior defects of the atrial septum (1,2). Figure 1 Transesophageal echocardiography showing a posteroinferior interatrial connection (arrow) near the inferior vena cava (IVC) (A and B), and a small, separate patent foramen ovale (arrowhead) (C). LA Left atrium; RA Right atrium; RIPV Right inferior pulmonary ... Figure 2 Transesophageal echocardiography showing an intact muscular limbus (arrow) unrelated to the oval fossa and patent foramen ovale (arrowhead). IVC Inferior vena cava