Gräni Christoph, Stark Anselm W, Lo Rito Mauro, Frigiola Alessandro, Siepe Matthias, Tchana Bertrand, Cipriani Alberto, Zorzi Alessandro, Pergola Valeria, Crea Domenico, Sarris George, Protopapas Elephterios, Sirico Domenico, Di Salvo Giovanni, Pegoraro Cinzia, Sarto Patrizio, Francois Katrien, Frigiola Alessandra, Cristofaletti Alessandra, Accord Ryan E, Gonzalez Rocafort Alvaro, Debeco Geoffroy, Padalino Massimo
Department of Cardiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Department of Congenital Cardiac Surgery, IRCCS Policlinico San Donato, Milan, Italy.
Interdiscip Cardiovasc Thorac Surg. 2024 May 2;38(5). doi: 10.1093/icvts/ivae074.
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 centres.
EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023.
A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (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, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases.
Currently, no uniform agreement exists among European centres 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.
冠状动脉异常起源（AAOCA）是一组临床表现各异的罕见先天性心脏缺陷。AAOCA患者的终身风险尚不清楚，迫切需要多中心注册数据来调整当前建议并指导最佳患者管理。欧洲AAOCA注册研究（EURO - AAOCA）旨在评估各中心在AAOCA管理方面的差异。
EURO - AAOCA是一项前瞻性多中心注册研究，包括13个欧洲中心。在此，我们评估了2019年1月至2023年6月期间各中心在临床表现和管理、治疗决策及手术结果方面的差异。
共纳入262例AAOCA患者，中位年龄为33岁（12 - 53岁），呈双峰分布。139例（53.1%）有症状，其中胸痛（n = 74，53.2%）是最常见的主诉，其次是晕厥（n = 21，15.1%）。7例（5%）患者发生心肌梗死，2例（1.4%）发生心脏骤停。右冠状动脉异常起源最常见（150例，57.5%），其次是左冠状动脉异常起源51例（19.5%），回旋支冠状动脉异常起源20例（7.7%）。不同年龄组和各中心之间在诊断方面存在显著差异。74例（28.2%）患者接受了手术，无手术死亡；10例（3.8%）发生轻微术后并发症。
目前，欧洲各中心在AAOCA变异型的诊断方案和临床管理方面尚未达成统一共识。尽管手术在AAOCA中是一种安全的操作，但未来的长期结果数据有望阐明如何最佳地决定对患者进行血运重建与保守治疗的最佳选择。
