A Novel Approach to Surgical Treatment of Scimitar Syndrome: Relocation of the Anomalous Pulmonary Vein and Intra-Atrial Baffle Rerouting

(Department of Cardiovascular Surgery, Tokyo Metropolitan Childrenfs Hospital, Kiyose, Japan)

Yoshimasa Uno Takaaki Suzuki Kentaro Hotoda
Osamu Ishida Toyoki Fukuda
Scimitar syndrome is a rare congenital cardiac anomaly with anomalous right pulmonary veins draining to the inferior caval vein. Currently, it is widely accepted that there are 2 forms of presentation with either an infantile manifestation or an adult form. Patients in the latter category are usually less severely affected and frequently asymptomatic on diagnosis. A 16-year-old boy who had been given a diagnosis of scimitar syndrome was observed for years because of his unwillingness to undergo surgery. However, since the latest catheter examination demonstrated an elevated pulmonary-to-systemic flow ratio of 2.39, he consented to undergo surgical treatment. Preoperative studies demonstrated an intact atrial septum and abnormal bronchial arborization of the right lung. Pulmonary angiography demonstrated abnormal right pulmonary veins that converged to a single venous trunk, the so-called scimitar vein, and drained into the inferior caval vein at the level of diaphragma. Because of the morphological abnormalities including a wide distance between the pulmonary veno-caval junction and interatrial septum, counterclockwise rotation of the heart, and a small left atrium, surgical management was performed with a novel approach, consisting of relocation of the scimitar vein to the anterolateral wall of the right atrium, total excision of the oval fossa, and intra-atrial baffle rerouting with the pulmonary venous blood being conveyed to the left atrium through the atrial septal defect. During the cardiopulmonary bypass vacuum assisted venous drainage through a femoral venous cannula was highly effective to secure a clear operative field without occlusion of the inferior caval vein. Postoperative recovery was uneventful and the repeat Doppler echocardiography demonstrated an unobstructed flow through the baffle. This experience indicates that the above novel approach is a promising surgical option for the management of scimitar syndrome.
@Jpn. J. Cardiovasc. Surg. 36: 305-308 (2007)