title: Congenital circumflex artery-coronary sinus fistula in an adult female associated with severe mitral regurgitation and myelodysplasy--case report and review of the literature.
abstract: BACKGROUND
  Coronary artery fistulas are uncommon anomalies. They occur in 0.1-0.2% of patients undergoing coronary arteriography. The origin of the fistulas is the right coronary artery followed by the left anterior descending and lastly by the circumflex artery (17%). Termination into the right heart side occurs in 90% of cases. Termination into the coronary sinus is rare in 3% of cases. Circumflex artery-coronary sinus fistulas are even rarer.
  
  
  METHODS
  A single case report and literature review between 1993 and 2007.
  
  
  RESULTS
  We describe a 76-year-old female, who was analyzed for dyspnea on exertion (DOE) and chronic fatigue, with known myelodysplastic syndrome and an aneurysmal circumflex coronary artery-coronary sinus fistulous connection associated with severe mitral regurgitation. Mitral valve replacement using a bioprosthesis was performed as well as ligation of the fistula. The postoperative course was complicated with cardiac tamponade, which was successfully drained.
  
  
  CONCLUSIONS
  Our patient presented with chronic fatigue and DOE and was found to have a coronary artery fistula and severe mitral regurgitation associated with known myelodysplasia. Conventional coronary angiography failed to demonstrate the entire fistula characteristics (origin, pathway, and outflow). Multidetector computed tomography was complementary to demonstrate the complex anatomy of the fistula. The fistula was surgically ligated in combination with mitral valve replacement. She remains well.
authors.affiliation: Hospital Group Twente, Department of Cardiology, Hengelo, The Netherlands. samsaid@home.nl
authors.forename: Salah A M
authors.initials: SA
authors.lastname: Said
authors.forename: Aize
authors.initials: A
authors.lastname: van der Sluis
authors.forename: Kenneth
authors.initials: K
authors.lastname: Koster
authors.forename: Hauw
authors.initials: H
authors.lastname: Sie
authors.forename: Ghada M M
authors.initials: GM
authors.lastname: Shahin
mesh_terms: D000368:Aged; D001164:Arteriovenous Fistula / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D001705:Bioprosthesis; D002305:Cardiac Tamponade / Q000209:etiology; D017023:Coronary Angiography / Q000379:methods; D054326:Coronary Sinus / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D003330:Coronary Vessel Anomalies / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D004417:Dyspnea / Q000209:etiology; D005221:Fatigue / Q000209:etiology; D005260:Female; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation; D006801:Humans; D008026:Ligation; D008944:Mitral Valve Insufficiency / Q000150:complications* / Q000601:surgery; D009190:Myelodysplastic Syndromes / Q000150:complications*; D011474:Prosthesis Design; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome