Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital anomaly, in which all the right pulmonary veins drain into the inferior vena cava. In this study, we review the diagnostic features, clinical management, and surgical strategy in the Scimitar syndrome and discuss the significance of new generation diagnostic imaging methods for this rare anomaly.

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BackgroundRedo-sternotomy for mitral valve (MV) surgery may be complex and attendant complications can be avoided using anterolateral right thoracotomy, deep hypothermia (20°C, nasopharyngeal) with low flow cardiopulmonary perfusion. Video-assisted minithoracotomy technique is a further improvement.MethodsWe performed 20 consecutive MV operations in patients with previous cardiac surgery using video-assisted right minithoracotomy, femoro-femoral bypass, deep hypothermia, low flow cardiopulmonary bypass without aortic cross-clamping. The mean follow-up was 30 ± 17.8 mo. Data is presented as the mean ± standard deviation of the mean.ResultsThere were 11 males and 9 females (age, 62.3 ± 12.1; ejection fraction 50.1 ± 11.2). Operations included MV replacement (n = 11), MV repair (n = 5), and MV re-replacement (n = 4). There were no hospital deaths, and the mean hospital stay was 8 ± 2.9 days. There were no postoperative strokes or need for mechanical circulatory support. The mean cardiopulmonary bypass time was 152 ± 28 minutes. Two patients (10%) required inotropic support beyond 24 hrs. All patients were free from inotropic support at 48 hours. The mean number of transfused red cell units was 2.8 ± 0.8 (range, 2 to 4). One patient died in another institution six months postoperatively following surgery for acute type III aortic dissection. At 30 ± 17.8 months follow-up all patients were found to be in NYHA Class I or II.ConclusionsMinimally invasive video-assisted MV surgery using deep hypothermia, low-flow cardiopulmonary bypass without aortic clamping can result in excellent clinical outcomes in patients with previous cardiac surgery via a median sternotomy. This technique offers reproducible results, good myocardial protection (as evidenced by the low rate of inotropic support that patients needed postoperatively), and low rates of complications.