First report from the european registry for anomalous aortic origin of coronary artery (EURO-AAOCA)

Objectives: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centers. Methods: EURO-AAOCA is a prospective, multicentre registry including 13 european centers. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centers from 01/2019 to 06/2023. Results: 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. 139 (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, two (1.4%) with aborted sudden cardiac death. Right-AAOCA (R-AAOCA) was most frequent (150, 57.5%), followed by left-AAOCA (L-AAOCA) in 51 (19.5%), and circumflex-AAOCA (Cx-AAOCA) in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centers. 74 (28.2%) patients underwent surgery with no operative deaths; minor post-operative complications occurred in 10 (3.8%) cases. Conclusions: Currently no uniform agreement exists among european centers with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.