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......................................................... three levels: (i) patients who require care exclusively in a specialist centre, (ii) patients for whom shared care can be established with the appropriate general adult cardiac services, and (iii) patients who can be managed in non-specialist clinics (with access to specialized care if required). The proposed staff requirements for specialist centres are described in Table 5. The complexity of the heart defect should not be the only criterion to assign patients to a certain level of care. Although patients with complex defects can easily be assigned to a high level of care, even anatomically simple defects may require specialist care under certain circumstances [e.g. atrial septal defect (ASD) with pulmonary arterial hypertension (PAH)]. Therefore, it is recommended that all ACHD patients are seen once in a specialist centre, allowing ACHD specialists to determine the most appropriate level of care and follow-up intervals for each individual patient.8 Networks of specialist centres with general adult care should be established for each catchment area. Indeed, with the growing population of adults with CHD, more patients will first present to general cardiologists for acute conditions, such as arrhythmia, heart failure, or endocarditis. In such cases, general cardiologists should not delay treatment in haemodynamically unstable patients but ought to liaise with the patient’s ACHD centre immediately to discuss appropriate management strategies or transfer of the patient. Special attention is necessary for patients after Fontan correction presenting with arrhythmia, as even supraventricular arrhythmias are not well tolerated. Detailed advice on emergency care for ACHD patients will follow in a separate future position paper.

Transfer of adolescents to ACHD care is vital as they approach adulthood without gaps in care, and should be preceded by a preparatory transition phase with additional transition support that continues into early adulthood according to the needs of the patient.10 Transition requires a special healthcare organization.10,11 It is recommended that specialist ACHD centres have teams including specialist nurses, psychologists, and social workers given that anxiety, depression, or problems coping with the medical condition are well known concerns in adult patients with CHD.12 They also play a critical role in the transition process by taking over the transitional care of patients after the transfer from paediatric cardiology. Aspects that have to be addressed by allied health professionals include mental health, psychic well-being, and quality of life.12,13 During the ACHD

N Surgery should be considered for ARCAPA in asymptomatic patients with ventricular dysfunction, or myocardial ischaemia attributable to

coronary anomaly.

B) Anomalous aortic origin of a coronary artery (AAOCA) N 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. N Surgery should be considered in asymptomatic patients with AAOCA (right or left) and evidence of myocardial ischaemia. N Surgery should be considered in asymptomatic patients with AAOLCA and no evidence of myocardial ischaemia but a high-risk anatomy. N Surgery may be considered for symptomatic patients with AAOCA even if they have neither evidence of myocardial ischaemia nor high-

risk anatomy.

N Surgery may be considered for asymptomatic patients with AAOLCA without myocardial ischaemia and without high-risk anatomy when

they present at young age (<35 years).

N Surgery is not recommended for AAORCA in asymptomatic patients without myocardial ischaemia and without high-risk anatomy. New concepts

Naming (ACHD). Disease complexity classification. Staffing requirements for an ACHD expert centre. Emerging role of biomarkers in follow-up of ACHD. Detailed and specific recommendations on arrhythmia management. More specific and adjusted recommendations for PAH treatment. Recommendations on the use of anticoagulants. Consideration of ageing and advance care planning. Categories of high-risk pregnancies in accordance with the pregnancy guidelines. Expand Marfan syndrome section to aortopathies (and include HTAD, Turner syndrome, and bicuspid aortic disease). Emerging role of catheter intervention in ACHD.

6MWT = 6-minute walk test; AAOCA = anomalous aortic origin of a coronary artery; AAOLCA = anomalous aortic origin of the left coronary artery; ACAPA = anomalous coronary artery from the pulmonary artery; ACHD = adult congenital heart disease; AF = atrial fibrillation; ALCAPA = anomalous left coronary artery from the pulmonary artery; ARCAPA = anomalous right coronary artery from the pulmonary artery; AS = aortic stenosis; ASD = atrial septal defect; AV = atrioventricular; AVSD = atrioventricular septal defect; BNP = B-type natriuretic peptide; ccTGA = congenitally corrected transposition of the great arteries; CHD = congenital heart disease; CMR = cardiovascular magnetic resonance; EF = ejection fraction; HTAD = heritable thoracic aortic disease; IART = intraatrial reentrant tachycardia; ICD = implantable cardioverter defibrillator; LR = left-to-right; LV = left ventricle/ventricular; LVEF = left ventricular ejection fraction; LVESD = left ventricular end systolic diameter; LVOT = left ventricular outflow tract; LVOTO = left ventricular outflow tract obstruction; N = new recommendation; PAH = pulmonary arterial hypertension; PAH-CHD = pulmonary arterial hypertension associated with congenital heart disease; PAP = pulmonary artery pressure; PDA = patent ductus arteriosus; PFO = patent foramen ovale; PH = pulmonary hypertension; PR = pulmonary regurgitation; PVR = pulmonary vascular resistance; Qp:Qs = pulmonary to systemic flow ratio; R = revised recommendation; RA = right atrium/atrial; RL = rightto-left; RV = right ventricle/ventricular; RVEDVi = right ventricular end diastolic volume indexed; RVESVi = right ventricular end systolic volume indexed; RVOT = right ventricular outflow tract; RVOTO = right ventricular outflow tract obstruction; RVSP = right ventricular systolic pressure; SVT = supraventricular tachycardia; TGA = transposition of the great arteries; TGFBR = transforming growth factor beta receptor; TOF = tetralogy of Fallot; TPVI = transcatheter pulmonary valve implantation; TR = tricuspid regurgitation; TV = tricuspid valve; UVH = univentricular heart; VSD = ventricular septal defect; VT = ventricular tachycardia; WU = Wood units. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026