title: Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches.
abstract: OBJECTIVE
  Patients with a coronary artery arising from the wrong sinus are susceptible to ischemia and sudden death. Risk is higher when the artery courses interarterially--between the pulmonary artery and aorta--has an intramural course, or has an abnormal orifice. In single coronary ostium without intramural course, unroofing and coronary reimplantation are inappropriate, and coronary artery bypass grafting is suboptimal. For this variant, we have devised pulmonary artery translocation.
  
  
  METHODS
  A retrospective review of 18 patients undergoing repair between January 1999 and March 2005 was performed. Mean age was 8.1 years (range 6 weeks-16 years). All anomalous arteries coursed interarterially. Ten patients had a right coronary artery from the left coronary sinus; 8 had a left coronary artery from the right sinus. Eleven had an intramural course, and 4 had a single coronary ostium without an intramural course. Ten (56%) patients had symptoms: chest pain (9/10), syncope (3/10), or dyspnea (2/10). Repair was implemented by unroofing (n = 11), reimplantation (n = 3), or pulmonary artery translocation (1 lateral, 3 anterior). All patients were followed up clinically and echocardiographically.
  
  
  RESULTS
  At a mean of 2.2 years (2 weeks-5 years), there was no mortality. Symptoms improved and function remained normal in all but 1 patient. He had sustained multiple infarcts in the anomalous artery's distribution and required transplantation despite repair.
  
  
  CONCLUSIONS
  Repair is indicated in all patients with coronary insufficiency and in asymptomatic patients with high-risk morphologic abnormalities. We recommend unroofing when an intramural component (or slit-like orifice) is present, reimplantation for separate ostia without an intramural course, and pulmonary artery translocation for single ostium without an intramural course. Coronary artery bypass grafting is thus avoided.
authors.affiliation: Department of Pediatric Cardiac Surgery, Stanford University Medical Center, Stanford, Calif, USA. rgulati@alumni.duke.edu
authors.forename: Rajeev
authors.initials: R
authors.lastname: Gulati
authors.forename: Vadiyala Mohan
authors.initials: VM
authors.lastname: Reddy
authors.forename: Casey
authors.initials: C
authors.lastname: Culbertson
authors.forename: Gregory
authors.initials: G
authors.lastname: Helton
authors.forename: Sam
authors.initials: S
authors.lastname: Suleman
authors.forename: Olaf
authors.initials: O
authors.lastname: Reinhartz
authors.forename: Norman
authors.initials: N
authors.lastname: Silverman
authors.forename: Frank L
authors.initials: FL
authors.lastname: Hanley
mesh_terms: D000293:Adolescent; D013504:Cardiovascular Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies