Cardiology Practice

Clinical Characteristics and Outcomes of Anomalous Aortic Origin of the Left Coronary Artery Subtypes in Children

Article Impact Level: HIGH
Data Quality: STRONG
Summary of Circulation: Cardiovascular Interventions, 16(5). https://doi.org/10.1161/CIRCINTERVENTIONS.122.012635
Dr. Tam T. Doan et al.

Points

  • Anomalous aortic origin of the left coronary artery (AAOLCA) in children poses a rare but significant risk of sudden cardiac death.
  • A prospective study analyzed the clinical characteristics and outcomes of three AAOLCA subtypes.
  • The study enrolled 56 patients, with group 1 (interarterial course) being the most common subtype.
  • Inducible ischemia was observed in all three subtypes, while aborted sudden cardiac deaths were more frequent in interarterial AAOLCA cases.
  • Surgical intervention was recommended for most patients, resulting in improved outcomes and freedom from symptoms during follow-up.

Summary

Anomalous aortic origin of the left coronary artery (AAOLCA) is a rare but potentially life-threatening condition in children, with interarterial AAOLCA presenting the highest risk of sudden cardiac death. This research aimed to analyze the clinical features and outcomes of three AAOLCA subtypes. The study enrolled 56 patients below 21 years old, categorized into three groups based on the AAOLCA subtype. Group 1 comprised AAOLCA originating from the right aortic sinus with an interarterial course, group 2 included AAOLCA with an intraseptal course, and Group 3 consisted of AAOLCA originating between the left and noncoronary aortic sinus.

Computed tomography angiography was used to assess the anatomical details of the patients. Provocative stress testing, including exercise stress testing and stress perfusion imaging, was conducted for patients over eight years old or younger with concerning symptoms. Surgery was recommended for patients in group 1 and in select cases for groups 2 and 3.

The results showed that the majority of patients in group 1 exhibited an intramural course (93%), compared to group 3 (56%) and group 2 (10%). Among the enrolled patients, 13% had presented with aborted sudden cardiac death, with group 1 accounting for six out of the seven cases and group 3 accounting for the remaining case involving cardiogenic shock. Provocative testing revealed inducible ischemia in 33% of the patients, distributed across all three AAOLCA subtypes. Surgery was recommended for 56% of the patients, with the highest rate in group 1 (93%). Surgery was successfully performed in 25 patients, resulting in asymptomatic conditions and freedom from exercise restrictions during a median follow-up period of 4 years.

In conclusion, this study demonstrates the presence of inducible ischemia in all three AAOLCA subtypes, with interarterial AAOLCA (group 1) showing a higher incidence of aborted sudden cardiac death. Additionally, AAOLCA cases with left/nonjuxtacommissural origin and the intramural course also carry a significant risk of aborted sudden cardiac death and cardiogenic shock. A systematic approach is crucial for accurate risk stratification of this patient population.

Link to the article: https://www.ahajournals.org/doi/10.1161/CIRCINTERVENTIONS.122.012635

References

Doan, T. T., Wilkes, J. K., Reaves O’Neal, D. L., Bonilla-Ramirez, C., Sachdeva, S., Masand, P., Mery, C. M., Binsalamah, Z., Heinle, J. S., & Molossi, S. (2023). Clinical presentation and medium-term outcomes of children with anomalous aortic origin of the left coronary artery: High-risk features beyond interarterial course. Circulation: Cardiovascular Interventions, 16(5). https://doi.org/10.1161/CIRCINTERVENTIONS.122.012635

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