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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course

Peter C. Frommelt, MD, FACC^*,*, Michele A. Frommelt, MD, FACC^*, James S. Tweddell, MD and Robert D. B. Jaquiss, MD

^* Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
Department of Cardiothoracic Surgery, Children’s Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

Manuscript received November 20, 2002; revised manuscript received January 12, 2003, accepted January 24, 2003.

^* Reprint requests and correspondence: Dr. Peter C. Frommelt, Children’s Hospital of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, Wisconsin 53226, USA.
pfrom{at}mcw.edu

OBJECTIVES: In this study, we sought to describe the mode of presentation, anatomic features, diagnostic techniques, and surgical outcome in a group of patients with anomalous origin of a coronary artery from the opposite sinus with an interarterial course between the great arteries (AOCA).

BACKGROUND: Anomalous origin of a coronary artery from the opposite sinus with an interarterial course is associated with myocardial ischemia and sudden cardiac death, particularly in adolescents and young adults.

METHODS: The cardiology database at Children’s Hospital of Wisconsin was reviewed to identify all patients diagnosed with AOCA.

RESULTS: From September 1997 to August 2002, 10 patients were identified with AOCA; all were children/adolescents (age range, 3 months to 20 years; weight range, 4.7 to 72 kg), and nine were diagnosed prospectively by transthoracic echocardiography (TTE). Symptoms of cardiac ischemia initiated investigation in 4/10 patients at a mean age of 16 ± 2.8 years; the other six had TTE for suspected congenital heart disease/musculoskeletal chest pain. The left coronary artery originated from the right sinus in six patients, and the right coronary artery originated from the left sinus in four patients. An intramural course of the AOCA within the anterior aortic wall was found in 9/10 patients and was reliably identified by TTE; the other patient had an intramyocardial course of the anomalous coronary. Surgical repair was performed in 8/10 patients at a mean age of 13 ± 4.7 years. Unroofing of the intramural portion of the AOCA to relocate the ostia in the appropriate sinus was successfully performed in seven patients. All patients status post unroofing were asymptomatic with patent coronary flow by Doppler and normal exercise treadmill testing at a median follow-up interval of 1.5 years.

CONCLUSIONS: Anomalous origin of a coronary artery from the opposite sinus with an interarterial course is frequently characterized by an intramural course, which can be prospectively identified by TTE. Unroofing the intramural segment without bypass grafting can reliably repair the intramural form of AOCA.

Abbreviations and Acronyms   AOCA   anomalous origin of a coronary artery from the opposite sinus with an interarterial course   ETT   exercise treadmill testing   TTE   transthoracic echocardiography   VSD   ventricular septal defect

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