• Simon Schwill, Jennifer Del Prete, Denton A Cooley, and David L S Morales.
• Department of Cardiovascular Surgery, Texas Heart Institute, St. Luke's Episcopal Hospital, Houston, Texas 77030, USA.
• Tex Heart Inst J. 2010 Jan 1;37(3):358-60.

AbstractScimitar syndrome is a rare but serious congenital condition that consists of anomalous pulmonary venous drainage of the right lung to the inferior vena cava. The appearance on chest radiography resembles a curved Turkish sword, or scimitar. Scimitar syndrome is associated with other anomalies, including hypoplasia of the right lung, dextroposition of the heart, anomalous systemic arterial supply to the right lung, and atrial septal defect. Clinical manifestations in infants include severe tachypnea and cyanosis. Adult patients may present with dyspnea and increased fatigue. Few adult cases have been reported in the medical literature. Classical surgical repair involves diversion of the scimitar venous flow into the left atrium with a baffle, requiring the use of cardiopulmonary bypass and deep hypothermic circulatory arrest. Herein, we report the case of a 42-year-old woman with 2 scimitar veins who underwent corrective surgery at our center without the use of cardiopulmonary bypass. We also comment on the importance of a patient's lung hypoplasia in the decision to repair the defect through a right thoracotomy.

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