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............................................................................................................................................................................. incompetence and arrhythmias are common problems, but with increasing age arrhythmias and HF predominate. In master athletes, problems related to previous corrective or palliative surgery become prevalent. These include cardiac arrhythmias, systemic ventricular dysfunction, valvar incompetence, and prosthetic conduit obstruction. Redo valve or conduit surgery and arrhythmia ablation due to re-entry arrhythmias (secondary to surgical scar) are frequent in this age group. In addition, with increasing age, acquired CVD may develop.581,582 This is of particular relevance in the assessment of the master athlete with CHD.

5.7.5 Sudden death during sport SCD in CHD is rare (<0.1% per year)583 and only 8% of deaths occur during exercise.584 Many complex CHD patients at highest risk of SCD have reduced exercise tolerance and cannot participate in significant athletic activity. However, some diagnostic groups, e.g. post tetralogy of Fallot repair, carry a risk of SCD but can still compete in elite sport.585 Other high-risk congenital lesions such as anomalous coronary origin, mitral valve prolapse, and aortopathy are described in sections 5.1, 5.3, and 5.4, respectively. In a large population-based study of SCD in CHD patients under 35 years, 87% of SCD was due to a presumed arrhythmia and 41% occurred in patients with undiagnosed CHD.586Activity-related SCD was more common in the undiagnosed group (18% vs. 4%), which supports the case for preparticipation athletic screening.586 It is not known whether exercise intensity is a risk factor for SCD in CHD. There is reasonable evidence that moderate to vigorous exercise is safe in most ACHD patients even when symptomatic (NYHA IIIII).576,587,588 However, cardiac arrhythmias are a common cause of hospital admission in ACHD and extreme exercise may expose a latent arrhythmic substrate.589,590

5.7.6 Exercise in athletes with congenital heart disease: current guidelines and recommendations Regular structured exercise is safe and effective therapy for most patients with CHD. This is true for most diagnostic groups, including symptomatic patients, and includes aerobic and strength-based exercise.588,591596 Exercise intolerance in CHD is a strong predictor of both outcome and SCD.576 Specific precautions are necessary in extreme environments including underwater sports and this is considered below (see Supplementary Data, section 4). This is particularly true if there is a potential for a right to left shunt. Guidelines are available for exercise assessment and prescription in both children and adults with CHD, although these are not specifically designed for the athlete.597,598 The paediatric guidelines are based primarily on the underlying anatomical diagnosis, but the teenage and young adult CHD guidelines have taken a functional approach based on underlying haemodynamics and arrhythmia risk. This latter approach is better suited for assessment of the ACHD athlete (Figure 9).

5.7.7 Assessment of the athlete with congenital heart disease The guidelines for exercise prescription in adolescents and adults with CHD use a structured methodology described by Budts et al.597 This can be modified for use in the assessment of athletes with CHD. Stage 1. A full history and physical examination are carried out. This should include details of underlying CHD diagnosis, any transcatheter or surgical interventions, current medications and CV symptoms (at rest and on exercise). Attention should be paid to any associated non-cardiac diagnoses including pulmonary dysfunction. A full exercise and sports participation history should be taken including precise details of current training schedule and any dietary supplements. Details of the planned or current sports activity should be established to include an assessment of the static component and intensity as described in section 4.1 (see Figure 2). If necessary, advice should be sought from a sports fitness coach or sports medicine specialist.

Stage 2. The following five baseline parameters should then be evaluated597 (Table 16). (1) Ventricular function Assessment of ventricular function can usually be achieved using echocardiography. The aim is to establish whether function is reduced (EF < 55%), and if so, is it mild (4555%), moderate (3045%), or severe (<30%). This is used for baseline assessment and subsequent monitoring of the effects of exercise training. Echocardiography can also evaluate inflow and outflow abnormalities, which may become more severe during acute exercise (e.g. LV outflow obstruction or systemic atrioventricular valve regurgitation). CMR scanning may be a preferable modality in complex disease. This has the additional benefit of evaluating intracardiac scar, which may inform the assessment of arrhythmia risk.599,600 (2) Pulmonary artery pressure Pulmonary hypertension (PH) is diagnosed when the mean PAP is >20 mmHg.601 PH may occur in the context of a chronic leftright shunt (e.g. atrial septal defect, ventricular septal defect, patent ductus arteriosus) that allows unrestricted volume/pressure overload. Eventually this can result in supra-normal PAP with reversal of shunting and elevated pulmonary vascular resistance (Eisenmenger syndrome). An increased RV afterload limits the ability to increase cardiac output by increasing stroke volume and can impair LV function through disruption of normal RVLV interaction. Few CHD patients with significant PH will take part in competitive sports due to reduced exercise capacity. However, some athletes with CHD will have mild elevation of pulmonary vascular resistance that may be exacerbated by factors such as altitude training.602 PAP rises during exercise. This rise is accentuated with increasing age and may be exaggerated in CHD athletes due to elevated pulmonary vascular resistance.603 In addition, valvar pulmonary stenosis or distal stenoses in branch pulmonary arteries may cause exercise-related RV hypertension. The non-invasive assessment of PH can be difficult and full assessment guidelines have been published.604,605 Further information can be gained by CPET and a VO2 of >25.2 mL/min/kg makes significant PH unlikely.606 As PH can also be a late postoperative complication after surgical correction of CHD607 the assessment of PAP should be part of every echocardiographic examination in athletes with CHD. Although exercise training is generally regarded as safe in PH, competitive sport is not recommended.1,608 (3) Aortic assessment Many CHD patients are at risk of aortic dilatation, in particular, patients with tetralogy of Fallot, coarctation of the aorta and certain Downloaded from academic.oup.com/eurheartj/article/42/1/17/5898937 by guest on 07 October 2026