Adult Scimitar Syndrome: A surgical approach

Abstract

Background

Scimitar Syndrome is a rare congenital condition characterised by partial or total anomalous pulmonary venous drainage of the right lung. We present an adult case of Scimitar Syndrome that was managed with an intra-atrial baffle repair.

Case Description: The patient was a 52 year-old lady who had symptoms of increasing dyspnoea and fatigue with a long-term history of poor exercise tolerance. Several investigations such as a chest X-ray, echocardiogram and coronary angiogram confirmed the diagnosis of Scimitar syndrome with significant left to right shunt. The operation proceeded under cardiopulmonary bypass with a short period of hypothermic circulatory arrest. Postoperative recovery was uneventful and a follow-up echocardiogram confirmed the correction of the condition.

Discussion: There are many alternative and novel surgical techniques developed which include the reimplantation of the scimitar vein into the right atrium with the creation of a baffle through an ASD, direct anastomosis of the divided scimitar vein to the left atrium and many others. However, none of them have outcomes which are proven to be better than the technique we chose.

Conclusion: We utilised a well-recognised technique for a patient that had indications for surgical repair and this resulted in a good prognosis.