Schwill Simon, Del Prete Jennifer, Cooley Denton A, Morales David L S
Department of Cardiovascular Surgery, Texas Heart Institute, St. Luke's Episcopal Hospital, Houston, Texas 77030, USA.
Tex Heart Inst J. 2010;37(3):358-60.
Scimitar 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.
弯刀综合征是一种罕见但严重的先天性疾病,其特征为右肺肺静脉异常引流至下腔静脉。胸部X线表现类似弯曲的土耳其剑,即弯刀。弯刀综合征常伴有其他异常,包括右肺发育不全、心脏右旋、右肺异常体循环动脉供血以及房间隔缺损。婴儿的临床表现包括严重的呼吸急促和发绀。成年患者可能出现呼吸困难和疲劳加重。医学文献中报道的成年病例较少。经典的手术修复方法是使用挡板将弯刀状静脉血流改道至左心房,这需要使用体外循环和深度低温循环停搏。在此,我们报告一例42岁女性患者,其有两条弯刀状静脉,在我们中心未使用体外循环进行了矫正手术。我们还讨论了患者肺发育不全在决定通过右胸切口修复缺损中的重要性。