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........................................................................................ common, but more malignant than the right coronary artery from the left sinus. High orifice, ostial stenosis, slit-like/fish-mouth-shaped orifice, acute-angle take-off, intramural course and its length, or interarterial course and hypoplasia of the proximal coronary artery have been associated with myocardial ischaemia and have all been proposed as risk factors.319322 Risk stratification must also include age (<35 years) and level of exercise (e.g. competitive sports). There is very limited evidence that surgery in asymptomatic middle-aged patients provides any survival benefit or modifies the SCD risk.323,324 4.17.1.3 Coronary artery fistulae A coronary artery fistula, whether congenital or acquired, is an abnormal connection between a coronary artery and cardiac chamber or vessel. Small fistulae have a good prognosis without treatment. Medium or large fistulae are associated with long-term complications (angina, myocardial infarction, arrhythmias, heart failure, and endocarditis). The presence of symptoms, complications, and a significant shunt are the main indications for percutaneous or surgical closure. 4.17.2 Diagnostic evaluation CCT is the preferred technique for the evaluation of high-risk anatomy, including features such as an intramural course and orifice anomalies (slit-like orifice, acute-angle take-off, orifice >1 cm above the sinotubular junction). Assessment of physical stress-induced ischaemia using advanced imaging modalities is the key to decision making. 4.17.3 Surgical treatment Indications for surgery are summarized in the Recommendations for the management of patients with anomalous coronary arteries table. 5 Quality indicators The overall aim of this new edition of the ESC Clinical Practice Guidelines for ACHD is to assist caregivers in their daily practice for the benefit of patients. In a next step, it is important to analyse the adaptation of, and the adherence to, these Guidelines in practice, a process that can be envisaged using Quality Indicators (QIs). QIs are sets of measures that enable the quantification of adherence to Guideline recommendations and provide a mechanism for measuring opportunities to improve cardiovascular care and outcomes.325 QIs show important differences from Clinical Practice Guidelines. For instance, the latter are recommendations for care to apply prospectively to individual patients, whereas QIs are applied retrospectively to a group of patients to assess if a care process was delivered or not.325 QIs are derived from evidence, feasible, concretely interpretable, and usable.326 The goal of QIs is to improve quality of health care, and they have been increasingly used by health authorities, professional organisations, healthcare payers, as well as the public.327329 The process of development and defining QIs for the management of specific ACHD items has been initiated during the Guideline writing process and results will be published later in a dedicated document. Recommendations for the management of patients with anomalous coronary arteries Recommendations Classa Levelb Non-pharmacological functional imaging (e.g. nuclear study, echocardiography, or CMR with physical stress) is recommended in patients with coronary anomalies to confirm/exclude myocardial ischaemia. I C Anomalous coronary arteries from the pulmonary artery Surgery is recommended in patients with ALCAPA. I C Surgery is recommended in patients with ARCAPA and symptoms attributable to anomalous coronary artery. I C Surgery should be considered for ARCAPA in asymptomatic patients with ventricular dysfunction, or myocardial ischaemia attributable to coronary anomaly. IIa C Anomalous aortic origin of the coronary artery Surgery is recommended for AAOCA in patients with typical angina symptoms who present with evidence of stress-induced myocardial ischaemia in a matching territory or high-risk anatomy.c I C Continued Surgery should be considered in asympto- matic patients with AAOCA (right or left) and evidence of myocardial ischaemia. IIa C Surgery should be considered in asymptomatic patients with AAOLCA and no evidence of myocardial ischaemia but a highrisk anatomy.c IIa C Surgery may be considered for symptomatic patients with AAOCA even if there is no evidence of myocardial ischaemia or highrisk anatomy.c IIb C Surgery may be considered for asymptomatic patients with AAOLCA without myocardial ischaemia and without high-risk anatomyc when they present at young age (<35 years). IIb C Surgery is not recommended for AAORCA in asymptomatic patients without myocardial ischaemia and without high-risk anatomy.c III C AAOCA = anomalous aortic origin of a coronary artery; AAOLCA = anomalous aortic origin of the left coronary artery; AAORCA = anomalous aortic origin of the right coronary artery; ALCAPA = anomalous left coronary artery from the pulmonary artery; ARCAPA = anomalous right coronary artery from the pulmonary artery; CMR = cardiovascular magnetic resonance. aClass of recommendation. bLevel of evidence. cHigh-risk anatomy includes features such as an intramural course and orifice anomalies (slit-like orifice, acute-angle take-off, orifice >1 cm above the sinotubular junction). Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026