A Novel Approach to Surgical Treatment of Scimitar Syndrome: Relocation of the Anomalous Pulmonary Vein and Intra-Atrial Baffle Rerouting / 日本心臓血管外科学会雑誌

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.

Successful Surgical Repair of Double-Outlet Right Ventricle in a Patient with Congenital Mitral Valve Stenosis / 日本心臓血管外科学会雑誌

A 2-year-old boy was admitted to our service with a diagnosis of double-outlet right ventricle (DORV) complicated by mitral valve stenosis. On echocardiographic measurement, the mitral annulus was 11mm (69% of normal) in diameter and 1.67cm²/m² in annular area. He underwent intraventricular tunnel repair and patch reconstruction of the right ventricular outflow tract under cardiopulmonary bypass. No attempt was made to operate on the mitral valve. DORV with mitral stenosis is a rare cardiac anomaly, the last 20 years only 21 cases have been reported, of which only 4 were cases with hypoplastic mitral rings. Although the patient made an uneventful recovery and remains well 5 months after surgery, a close observation is required for the mitral valve stenosis.

Four Cases of Partial Anomalous Pulmonary Venous Return with Intact Atrial Septum / 日本心臓血管外科学会雑誌

While atrial septal defect is a common associated feature of partial anomalous pulmonary venous return, atrial septum reportedly remains intact in the incidences ranging from 2.2 to 26.0%. We have experienced four cases of isolated partial anomalous pulmonary venous return without other cardiac anomalies including atrial septal defect. Entire right pulmonary veins returned to right atria in two of the cases. Remaining two cases were extremely rare in type. In one of those, right and left upper pulmonary veins returned to superior vena cava and left innominate vein, respectively, and entire left pulmonary veins returned to left innominate vein in the other. Three of the cases underwent complete correction with excellent results.