Inferior sinus venosus defect: echocardiographic diagnosis and surgical approach.
OBJECTIVE
We sought to define the inferior sinus venosus defect anatomically and document successful surgical approaches.


METHODS
We identified all patients previously given a diagnosis of an inferior sinus venosus defect at the Hospital for Sick Children, Toronto, Canada, between 1982 and 2005 by interrogating the cardiology and cardiac surgery databases. We included those having interatrial communications in which 1 or more of the right pulmonary veins drained to the inferior caval vein but retained connection with the left atrium, the rims of the oval fossa, and the walls of the coronary sinus, both being intact.


RESULTS
We identified 11 children who had an interatrial communication meeting the criteria for and undergoing surgical repair of an inferior sinus venosus defect. Median age was 1.2 years; 6 (55%) subjects were male, and none were cyanotic. Transthoracic echocardiographic analysis was performed preoperatively in all children, revealing right ventricular dilation in all. Surgical repair was accomplished with a pericardial patch. A complex baffle was needed in 3 children to maintain unobstructed inferior caval and pulmonary venous return. The echocardiographic diagnosis was complete in only 5 patients, but all diagnoses were correct since the year 2000. In all children the observations at surgical intervention showed that the defect was a venoatrial communication involving drainage of the right pulmonary veins to the inferior caval vein while retaining connection to the left atrium.


CONCLUSIONS
Transthoracic echocardiographic analysis should remain the modality of choice for diagnosis of the inferior sinus venosus defect. We report excellent surgical results with a patch or baffle, correctly redirecting the anomalous venoatrial connections.
Crystal
Matthew A
MA
Division of Cardiology, Department of Pediatrics, University of Toronto, the Hospital for Sick Children, Toronto, Ontario, Canada.
Al Najashi
Khaled
K
Williams
William G
WG
Redington
Andrew N
AN
Anderson
Robert H
RH
D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014682:Vena Cava, Inferior / Q000002:abnormalities* / Q000000981:diagnostic imaging