Siddiqui Faisal Mujib, Rubio Edmundo Raul, Patel Vishal M, Aziz Sameh, Ie Susanti
1 Department of Pulmonary and Critical Care, 2 Department of Radiology, Virginia Tech-Carilion School of Medicine, Roanoke, VA, USA.
Ann Transl Med. 2016 May;4(10):198. doi: 10.21037/atm.2016.05.29.
Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CxR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or Scimitar. A 27-year-old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. Her CxR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Chest computed tomography (CT) demonstrated that these opacities join to represent an anomalous vein draining into IVC. Furthermore, an anomalous systemic artery arising from the abdominal aorta was seen to supply the right lower lobe. The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births while nearly half of the patients remain asymptomatic with some initially being misdiagnosed as dextrocardia, such as in our case. Correctly diagnosing these patients is of paramount importance as some can develop severe pulmonary hypertension and right ventricular failure. In turn, close ongoing echocardiographic monitoring can help identify those that may benefit from surgical interventions to prevent them from developing these complications.
弯刀综合征的特征是静脉回流异常,胸部X线片(CxR)具有特征性表现,即异常静脉引流至下腔静脉(IVC)。这表现为一条与心脏右缘平行的曲线状不透光区,类似一把弯曲的剑或弯刀。一名有右位心病史的27岁白人女性在药物过量后入院。检查发现该女性意识模糊,有心动过速和右侧心音。她的胸部X线片显示心脏影像在右侧,心后区有两个曲线状不透光区。胸部计算机断层扫描(CT)显示这些不透光区相连,代表一条引流至下腔静脉的异常静脉。此外,还发现一条起自腹主动脉的异常体动脉为右下叶供血。该患者最终被诊断为弯刀综合征。这种综合征在每10万例活产中影响1 - 3例,近一半患者无症状,有些患者最初被误诊为右位心,就像我们这个病例。正确诊断这些患者至关重要,因为有些患者可能会发展为严重的肺动脉高压和右心室衰竭。反过来,持续密切的超声心动图监测有助于识别那些可能从手术干预中获益的患者,以防止他们出现这些并发症。