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.................................... care journey, advance care planning choices and strategies relating to end of life also require expert support.

3.3 Diagnostic work-up

Besides a thorough clinical evaluation, the medical history, including detailed information about palliative or reparative surgery and catheter-based interventions, is of critical importance in the work-up of ACHD patients. The aim of analysing patient history is to assess present and past symptoms, as well as to look for intercurrent events and any changes in medication. The most frequent symptoms reported by ACHD patients are exercise intolerance and palpitations. Self-estimated physical capacity corresponds poorly with objective measures of exercise capacity.14 Therefore, cardiopulmonary exercise testing (CPET) has gained importance for objective assessment of exercise intolerance in both apparently asymptomatic and symptomatic patients. In addition, the patient should be questioned about his/her lifestyle to detect progressive changes in daily activity in order to limit the subjectivity of symptom analysis. In symptomatic patients, alternative causes such as anaemia, depression, weight gain, and physical deconditioning, besides the congenital defect and its sequelae or residuae, should be kept in mind and further excluded if necessary.

Clinical examination plays a major role and includes careful evaluation of any changes in auscultation findings, blood pressure, or

Table 4 Classification of congenital heart disease complexity MILD: • Isolated congenital aortic valve disease and bicuspid aortic disease • Isolated congenital mitral valve disease (except parachute valve, cleft leaflet) • Mild isolated pulmonary stenosis (infundibular, valvular, supravalvular) • Isolated small ASD, VSD, or PDA • Repaired secundum ASD, sinus venosus defect, VSD, or PDA without residuae or sequellae, such as chamber enlargement, ventricular dysfunction,

or elevated PAP. MODERATE: (Repaired or unrepaired where not specified; alphabetical order) • Anomalous pulmonary venous connection (partial or total) • Anomalous coronary artery arising from the PA • Anomalous coronary artery arising from the opposite sinus • Aortic stenosis - subvalvular or supravalvular • AVSD, partial or complete, including primum ASD (excluding pulmonary vascular disease) • ASD secundum, moderate or large unrepaired (excluding pulmonary vascular disease) • Coarctation of the aorta • Double chambered right ventricle • Ebstein anomaly • Marfan syndrome and related HTAD, Turner Syndrome • PDA, moderate or large unrepaired (excluding pulmonary vascular disease) • Peripheral pulmonary stenosis • Pulmonary stenosis (infundibular, valvular, supravalvular), moderate or severe • Sinus of Valsalva aneurysm/fistula • Sinus venosus defect • Tetralogy of Fallot  repaired • Transposition of the great arteries after arterial switch operation • VSD with associated abnormalities (excluding pulmonary vascular disease) and/or moderate or greater shunt. SEVERE: (Repaired or unrepaired where not specified; alphabetical order) • Any CHD (repaired or unrepaired) associated with pulmonary vascular disease (including Eisenmenger syndrome) • Any cyanotic CHD (unoperated or palliated) • Double-outlet ventricle • Fontan circulation • Interrupted aortic arch • Pulmonary atresia (all forms) • Transposition of the great arteries (except for patients with arterial switch operation) • Univentricular heart (including double inlet left/right ventricle, tricuspid/mitral atresia, hypoplastic left heart syndrome, any other anatomic abnormality with a functionally single ventricle) • Truncus arteriosus • Other complex abnormalities of AV and ventriculoarterial connection (i.e. crisscross heart, heterotaxy syndromes, ventricular inversion).

ASD = atrial septal defect; AV = atrioventricular; AVSD = atrioventricular septal defect; CHD = congenital heart disease; HTAD = heritable thoracic aortic disease; LV = left ventricle/ventricular; PA = pulmonary artery; PAP = pulmonary artery pressure; PDA = patent ductus arteriosus; VSD = ventricular septal defect. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026