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............................................................................................................................................................................. excursion and mitral annular plane systolic excursion are still valid, especially in longitudinal follow-up. For left ventricular (LV) systolic function, 3D echocardiography, tissue Doppler imaging, and two-dimensional deformation imaging have proved to be robust tools and deserve to be integrated into clinical practice. Even accounting for newer techniques, echocardiography retains a key role in longitudinal follow-up assessment of systolic function of a right or single ventricle, although for more accurate measurements additional imaging is often needed in the form of CMR.

3.3.2 Cardiovascular magnetic resonance imaging CMR has become an essential facility in the specialist unit. It enables 3D anatomical reconstruction, which is not restricted by body size or acoustic windows, and has rapidly improving spatial and temporal resolution.18 CMR requires a regular heart rhythm for optimal image quality, however, diagnostic CMR studies are often achievable even in patients with irregular heart rhythms [frequent ectopy or atrial fibrillation (AF)] and metallic artefacts. CMR is the gold-standard imaging method for quantification of volumes. It may be an alternative when echocardiography cannot be obtained with sufficient quality, or used as a second method when echocardiography measurements are borderline or ambiguous. Furthermore, the lack of radiation makes it a useful tool when serial evaluations are needed (e.g. for monitoring aortic dimensions). CMR allows calculation of systemic and pulmonary blood flow in patients with multiple sources of blood supply and, in combination with invasive catheterization, of pulmonary vascular resistance (PVR). Tissue characterisation for myocardial fibrosis is a unique capability of CMR. Late gadolinium enhancement CMR for focal fibrosis and interstitial fibrosis T1 mapping imaging are increasingly being applied in ACHD for their potential diagnostic and prognostic value. However, large CHD lesion-specific studies to determine if they predict survival are ongoing. To mitigate the low risk of nephrogenic systemic fibrosis, gadolinium contrast should be avoided in patients with a low glomerular filtration rate (<30 mL/min/1.73 m2). It is therefore recommended that creatinine levels are checked prior to CMR. Even though clinical repercussions have not yet been seen, long-term brain deposits of gadolinium  regardless of renal function  raised concerns about cumulative lifetime doses in CHD patients who undergo serial CMR from a young age. It is preferred, therefore, that gadolinium is selectively administered in specialist centres using a macrocyclic, rather than linear chelated, gadolinium contrast, which presents a decreased risk at the lowest dose to achieve image enhancement.19 Adults with CHD with conventional pacemakers (PM) and defibrillators (ICD) can undergo CMR within guidelines where local support is available.20 3D CMR imaging can be integrated into electrophysiology (EP) procedures to inform and guide them. 3D CCT and CMR reconstructions can also be used for virtual reality rehearsal of interventions or planning from patient-specific 3D prints.

Indications for CMR are summarized in Table 6.

3.3.3 Cardiovascular computed tomography CCT has high spatial resolution and rapid acquisition time; it is particularly relevant for imaging the great vessels, coronary arteries, and collateral arteries, and for parenchymal lung disease (Table 6). In many institutions, CCT is the preferred imaging modality in planning for transcatheter valve implantation. Ventricular size and function can be assessed, with inferior temporal resolution compared with CMR, typically with an increased radiation dose, and is therefore not used serially for this indication. Recent rapid developments have substantially reduced the amount of radiation exposure, achieving <5 mSv for a combined CCT coronary, pulmonary, and aortic angiogram. For ACHD patients, this has made CCT more attractive for specific indications such as assessment, in particular for coronary artery pathology, and detailed assessment of collaterals.21

CCT is particularly useful in emergency settings including dissection, pulmonary embolism, and paravalvular abscess in the setting of endocarditis, where it may have advantages over echocardiography and CMR due to being less susceptible to prosthetic valve artefact. In patients with prosthetic valves (in situ >3 months), fluorine-18fluorodeoxyglucose positron emission tomography/computed tomography is useful for early diagnosis of inflammation and infection at the site of the valve, and also for identifying infection at secondary sites.22

3.3.4 Cardiopulmonary exercise testing Formal exercise testing has an important role in the CHD population, in which quality of life and functional capacity are key measures of the success of intervention. CPET, including assessment of objective exercise capacity (peak oxygen consumption), ventilatory efficiency [ventilation to carbon dioxide output (VE/VCO2) slope], chronotropic and blood pressure response, as well as exercise-induced arrhythmia and desaturation, gives a broader evaluation of functional capacity and physical fitness, and has endpoints which correlate well with morbidity and mortality in ACHD patients.23 Serial exercise testing should therefore be a part of long-term follow-up protocols. It plays an important role in the timing of interventions and reinterventions. CPET is also a useful tool for recommending the intensity of physical activity based on an individualized exercise prescription.24 The 6-minute walk test (6MWT) is another simple test for quantification of exercise capacity; it relates to outcome in patients with PAH.

3.3.5 Cardiac catheterization Cardiac catheterization is mainly reserved for resolution of specific anatomical and physiological questions, or for intervention. Indications include assessment of PVR, ventricular diastolic function (including constrictive and restrictive physiology), pressure gradients, shunt quantification, coronary angiography, and evaluation of extracardiac vessels such as aortic pulmonary collateral arteries when noninvasive evaluation leaves uncertainty. In shunt lesions with Doppler echocardiographic signs of PH, catheterization  including testing of vasoreactivity  remains essential in the decision on shunt closure. Inhaled nitric oxide is the most widely used agent for this purpose. Estimation of PVR in shunt lesions requires accurate calculation of pulmonary flow using the Fick principle. This method with measured oxygen consumption allows the most accurate cardiac output quantification. Before surgery, coronary artery imaging should be performed (by CCT or invasive coronary angiography) in men >40 years of age, postmenopausal women, and patients with signs of, or one or more risk factors for, coronary artery disease (CAD).25

3.3.6 Biomarkers Different classes of biomarkers have been reported to be associated with adverse events in the CHD population, including Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026