title: Two scimitar veins in an adult: repair through a right thoracotomy without cardiopulmonary bypass.
abstract: 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.
authors.affiliation: Department of Cardiovascular Surgery, Texas Heart Institute, St. Luke's Episcopal Hospital, Houston, Texas 77030, USA.
authors.forename: Simon
authors.initials: S
authors.lastname: Schwill
authors.forename: Jennifer
authors.initials: J
authors.lastname: Del Prete
authors.forename: Denton A
authors.initials: DA
authors.lastname: Cooley
authors.forename: David L S
authors.initials: DL
authors.lastname: Morales
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D002315:Cardiopulmonary Bypass; D017023:Coronary Angiography; D004417:Dyspnea / Q000209:etiology; D005221:Fatigue / Q000209:etiology; D005260:Female; D006801:Humans; D008026:Ligation; D008168:Lung / Q000002:abnormalities / Q000098:blood supply*; D010492:Pericardiectomy; D011667:Pulmonary Veins / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D012587:Scimitar Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D013908:Thoracotomy; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures; D014682:Vena Cava, Inferior / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*