P2.B025: Anomalous Aortic Origin of a Coronary Artery: Characteristics of a Pediatric Population Requiring Repeat Coronary Intervention

Background: Anomalous aortic origin of a coronary artery (AAOCA) is a rare cardiac anomaly associated with sudden cardiac death (SCD). Expert opinion recommends surgery for certain high-risk anatomic variants. Although surgical repair is largely regarded as safe, with low incidence of morbidity and mortality, there is a rare long-term risk of requiring coronary reoperation. We sought to describe characteristics of pediatric patients requiring coronary reoperation.

Methods: Institutional electronic medical record was queried from January 2001 to April 2024 to identify patients with a diagnosis of AAOCA and build a registry inclusive of all ages. We reviewed the characteristics of patients who underwent coronary reoperation, excluding aortic insufficiency and other non-coronary indications.

Results: 322 total patients with AAOCA were identified, with 219 pediatric patients. 99 pediatric patients underwent unroofing or reimplantation surgery. Of these, 49.5% had an anomalous left coronary artery (ALCA), 50.5% an anomalous right coronary artery (ARCA). Surgical patients had a median follow up time of 1.5 years (IQR 6d – 4.5y). In this time period, 5 coronary reoperations (2 in males) were performed, 4 ALCA and 1 ARCA. Patients who underwent reoperation had a median age at initial operation of 12.8 years. The time to reoperation varied between 7 months to 7.5 years (mean 4.9y, SD 2.6y). Two patients presented after aborted SCD events, two patients had recurrence of chest pain, and one patient was asymptomatic, but restenosis was found at routine surveillance.
In three patients, echocardiogram demonstrated accelerated flow in the repaired coronary artery, suggesting stenosis. Pre-operative cardiac computed tomography angiography suggested ostial stenosis of the repaired anomalous coronary in 4 patients, and narrowing with an intramural segment in 1 patient. During reoperation, 3 patients had ostial stenosis and an intramural segment and underwent repeat unroofing of this region. One patient had only stenosis from fibrosis; the fibrotic tissue was shaved off. In the last patient, the right-left commissure was causing the stenosis. The left coronary artery was unroofed and a wedge of thickened tissue was removed. Since reoperation, 1 patient is asymptomatic, 2 patients have been lost to follow up, one patient has new palpitations undergoing evaluation and one patient continues to have intermittent chest pain of noncardiac etiology.

Conclusion: Although rare, there remains a risk of residual or recurrent ostial stenosis from fibrosis or scar after surgical correction for AAOCA. Although the majority of patients needing reoperation had ALCA, given the small sample size, anatomic characteristics that put patients at highest risk remain difficult to delineate. Patients who undergo surgical intervention for AAOCA require interval follow-up throughout life with both symptom assessment and imaging. Further multi-institutional, long term data is required to delineate those patients at highest risk for complications and optimal follow-up intervals.