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J Thorac Cardiovasc Surg 2009;137:1349-1355
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Inferior sinus venosus defect: Echocardiographic diagnosis and surgical approach

^a Division of Cardiology, Department of Pediatrics, University of Toronto, the Hospital for Sick Children, Toronto, Ontario, Canada
^b Division of Cardiology, Adult Congenital Cardiac Center, University of Toronto, University Health Network, Toronto, Ontario, Canada
^c Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, the Hospital for Sick Children, Toronto, Ontario, Canada
^d Division of Cardiology, Department of Paediatrics, Great Ormond Street Hospital, London, United Kingdom

Received for publication May 15, 2008; revisions received November 5, 2008; accepted for publication December 19, 2008.

^_* Address for reprints: Andrew N. Redington, MD, the Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8. (Email: andrew.redington{at}sickkids.ca).

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.