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J Thorac Cardiovasc Surg 2007;134:1171-1178
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches

^a Department of Pediatric Cardiac Surgery, Stanford University Medical Center, Stanford, Calif
^b Department of Pediatric Cardiology, Children’s Hospital Oakland, Oakland, Calif
^c Department of Pediatric Cardiology, Stanford University Medical Center, Stanford, Calif.

Received for publication June 22, 2006; revisions received January 29, 2007; accepted for publication February 14, 2007.

^_* Address for reprints: Rajeev Gulati, MD, Pomona Valley Hospital, 160 East Artesia Street, Suite 355, Pomona, CA 91767. (Email: rgulati{at}alumni.duke.edu).

Objectives: 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.

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Discussion
J. Thorac. Cardiovasc. Surg. 2007 134: 1176-1178. [Extract] [Full Text] [PDF]