title: Anomalous cord from the raphe of a congenitally bicuspid aortic valve to the aortic wall producing either acute or chronic aortic regurgitation.
abstract: OBJECTIVE
  This report calls attention to an unappreciated cause of both acute and chronic aortic regurgitation (AR).
  
  
  BACKGROUND
  Although stenosis develops in most patients with a congenitally bicuspid aortic valve (BAV), in others with this anomaly, pure AR (no element of stenosis) develops, some in the absence of infection or other clear etiology.
  
  
  METHODS
  We describe 5 men who underwent aortic valve replacement for pure AR associated with a BAV containing an anomalous cord attaching the raphe of the conjoined cusp near its free margin to the wall of the ascending aorta cephalad to the sinotubular junction.
  
  
  RESULTS
  Three of these 5 patients had a history of progressive dyspnea, and the anomalous cord, which was intact at operation, appeared to cause chronic AR by preventing proper coaptation of the 2 aortic valve cusps. The other 2 patients heard a "pop" during physical exertion and immediately became dyspneic, and at operation, the anomalous cord was found to have ruptured. Prolapse of the conjoined aortic valve cusp toward the left ventricular cavity resulted in severe acute AR.
  
  
  CONCLUSIONS
  This variant of the purely regurgitant BAV may cause either chronic AR (when the anomalous cord does not rupture) or acute severe AR (when the cord ruptures).
authors.affiliation: Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
authors.forename: Travis J
authors.initials: TJ
authors.lastname: Vowels
authors.affiliation: Department of Cardiothoracic Surgery, Baylor University Medical Center, Dallas, Texas.
authors.forename: Gonzalo V
authors.initials: GV
authors.lastname: Gonzalez-Stawinski
authors.affiliation: Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
authors.forename: Jong M
authors.initials: JM
authors.lastname: Ko
authors.affiliation: Division of Cardiothoracic Surgery, The George Washington University Medical Center and Veterans Affairs Medical Center, Washington, DC.
authors.forename: Gregory D
authors.initials: GD
authors.lastname: Trachiotis
authors.affiliation: Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
authors.forename: Brad J
authors.initials: BJ
authors.lastname: Roberts
authors.affiliation: Palmetto Cardiovascular and Thoracic Associates, Trident Medical Center, Charleston, South Carolina.
authors.forename: Charles S
authors.initials: CS
authors.lastname: Roberts
authors.affiliation: Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas; Department of Internal Medicine (Division of Cardiology), Baylor University Medical Center, Dallas, Texas; Department of Pathology, Baylor University Medical Center, Dallas, Texas. Electronic address: wc.roberts@baylorhealth.edu.
authors.forename: William C
authors.initials: WC
authors.lastname: Roberts
mesh_terms: D000208:Acute Disease; D000328:Adult; D000368:Aged; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D002908:Chronic Disease; D003937:Diagnosis, Differential; D017548:Echocardiography, Transesophageal; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008875:Middle Aged
keywords: AR; BAV; aortic regurgitation; bicuspid aortic valve; congenital heart disease