Outcomes after surgical management of anomalous aortic origin of a coronary artery (AAOCA): an up-to-date systematic review and meta-analysis.

Background: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital anomaly that represents a leading cause of sudden cardiac death (SCD) in children and young athletes. Risk is concentrated in specific anatomical phenotypes, particularly anomalous aortic origin of the left coronary artery from the right sinus (L-AAOCA), variants with interarterial and/or intramural proximal course, and anomalous coronary orifice (slit-like) and acute take-off angle. Despite advances in multimodal imaging, optimal risk stratification and management remain controversial, and no randomized trials comparing surgical and conservative strategies exist. This study aims to systematically evaluate contemporary evidence and quantitatively estimate pooled clinical outcomes to define procedural safety and durability. Methods: A systematic search of PubMed/MEDLINE, Embase, Scopus, and the Cochrane Library was performed from database inception to March 2026. Eligible studies enrolled patients with AAOCA and reported management strategies and clinical outcomes. Original surgical series with fewer than 10 operated patients were excluded from the quantitative synthesis. A qualitative synthesis and random-effects meta-analysis of proportions were conducted to estimate pooled surgical incidence outcomes. Subjective postoperative symptoms and objectively documented residual ischemia were analysed as separate endpoints. For each outcome, study-specific evaluable denominators were used when follow-up populations differed from the baseline surgical cohort. Results: Twenty studies were included in the updated synthesis, substantially expanding the contemporary surgical evidence base. Pooled perioperative mortality was 0.85% [95% confidence interval (CI): 0.46-1.37%; I Conclusions: This up-to-date systematic review and meta-analysis found low perioperative mortality and favorable mid-term outcomes after AAOCA repair, although reintervention and postoperative AR remain relevant and vary by surgical technique. Persistent symptoms exceeded objective residual ischemia, indicating that symptoms are not a direct surrogate of residual ischemic risk. Conservative management showed low event rates in selected lower-risk cohorts, but cumulative risk may not be negligible. These findings support individualized, anatomy-driven management with careful technique selection and standardized long-term outcome reporting.