Competitive sports and congenital heart disease

Comparison of guidelines from various American and European scientific societies regarding participation in competitive sports among patients with congenital heart disease.

Physical activity provides well-known benefits, including improved mental health, reduced incidence of certain diseases, increased ability to perform basic daily activities, and enhanced muscular and bone strength. Among individuals with congenital heart disease (CHD), patients with better physical conditioning have demonstrated lower mortality rates; thus, physical activity in this population reduces mortality. Conversely, patients with CHD are not exempt from the adverse consequences of a sedentary lifestyle, notably metabolic syndrome. However, there is no zero-risk scenario for engaging in sports, especially competitive environments where exceeding individual limits is often the norm. 

This article aims to summarize and compare the recommendations from American guidelines, published in 2015 and endorsed by the American Heart Association (AHA) and the American College of Cardiology (ACC), with European guidelines, published in 2020 and supported by the European Association of Preventive Cardiology (EAPC), the European Society of Cardiology (ESC), and the Association for European Paediatric and Congenital Cardiology (AEPC).The 2015 AHA/ACC recommendations updated the previous 2005 guidelines, incorporating new sections on Marfan syndrome and sickle cell disease. These guidelines focus on patients aged 12 to 25 years but acknowledge applicability beyond this age range. Recommendations are based on various anatomical defects, with most evidence rated as Class C (expert opinions and consensus) and some specific defects rated as Class B (observational studies). 

The AHA/ACC evaluates sports based on two parameters: 

  • Dynamic component: involving continuous movement, such as running. 
  • Static component: involving minimal movement, such as archery. 

Each sport is categorized by dynamic intensity (A to C) and static intensity (I to III). 

Recommendations by anatomical defect: 

  • Simple shunts: Activity level depends on the hemodynamic significance of the shunt. For patients with pulmonary hypertension, sports are restricted to IA category. 
  • Pulmonary valve stenosis: The severity of stenosis determines the recommendation, with mild cases (<40 mm Hg) unrestricted, while moderate or severe cases (>60 mm Hg) are limited to IA and IB sports. Severe pulmonary insufficiency with right ventricular dilation also restricts activity to IA and IB. 
  • Aortic valve stenosis: Recommendations are based on mean and peak gradients. Mild cases (<25 mm Hg mean gradient) have no restrictions, moderate cases (25-40 mm Hg mean gradient) are limited to IA, IB, and IIA sports, and severe cases (>40 mm Hg mean gradient) are restricted to IA sports. 
  • Coarctation of the aorta: Recommendations for unrepaired cases depend on hemodynamic assessment and imaging. Repaired cases with normal hemodynamics and no dilation of the ascending aorta can participate in all sports except IIIA, IIIB, IIIC, and contact sports. Patients with aortic dilation are restricted to IA and IB sports. 
  • Pulmonary hypertension: Defined as mean pulmonary artery pressure >25 mm Hg, restricting sports to IA. Updated definitions lowering the threshold to 20 mm Hg were not included in the 2015 guidelines. 
  • Ventricular dysfunction after surgery: Patients with an ejection fraction (EF) of 40%-50% are limited to IA/B sports. EF <40% restricts activity to IA. 
  • Unrepaired cyanotic defects: Participation is restricted to IA sports, provided clinical stability. 
  • Repaired tetralogy of Fallot: Patients without arrhythmias, right ventricular outflow obstruction, or reduced EF (>50%) have no restrictions. Otherwise, sports are limited to IA. 
  • Transposition of the great arteries: For atrial switch repairs, restrictions include no Class C or III sports due to arrhythmia and ventricular dysfunction risk. Patients with arterial switch repairs and no dysfunction have no restrictions; mild dysfunction limits sports to IA/B/C and IIA. 
  • Fontan palliation: Recommendations are individualized. Asymptomatic patients with good hemodynamics may participate in IA sports. 
  • Ebstein anomaly: Patients with mild/moderate tricuspid regurgitation and normal right ventricular size have no restrictions, while severe cases are limited to IA. 

The 2020 European guidelines shifted focus from anatomical defects to individualized assessment based on hemodynamic and electrophysiological parameters. This updated philosophy emphasizes maximizing safe participation rather than restriction. Targeted at patients over 16 years old, these recommendations are based on expert consensus (Class C evidence). 

Sports are categorized into four types: 

  1. High-power sports (e.g., sprinting, weightlifting). 
  2. Skill-based sports (e.g., golf, archery). 
  3. Mixed sports (e.g., basketball, soccer).
  4. Indurance sports (e.g., cycling, marathons). 

The five-step algorithm includes: 

  1. Comprehensive history and physical examination. 
  2. Hemodynamic and electrophysiological assessment at rest. 
  3. Assessment during exercise. 
  4. Recommendations based on total findings. 
  5. Follow-up after implementing recommendations. 

Ideally, a complete cardiopulmonary test should include: peak oxygen consumption, heart rate reserve, effective ventilation slope, gas exchange, ischemia, blood pressure, among others. If these data are not available, graded exercise tests should be used. After obtaining and analyzing all the metrics, a patient risk profile is created. The degree of restriction will be determined by the abnormal metric. 

As a side note, there are injuries that have their own recommendations in different guidelines: patients with automatic defibrillators, cardiomyopathies, congenital coronary anomalies, arterial hypertension and hereditary arrhythmias. Patients who take anticoagulants of any kind are advised not to participate in contact sports. Cyanotic patients, with unrepaired injuries or with pulmonary hypertension, are advised not to participate in sports at high or moderate altitude. 

COMMENTARY: 

The article by Shibbani et al. does a great job comparing the similarities and differences of the guidelines on both sides of the Atlantic. The lack of updating of the American guidelines leads to some inconsistencies such as the case of a patient with aortic stenosis, a mean pressure of 45 mm Hg where he would be restricted to class IA sport. This patient would not be offered balloon valvuloplasty because a peak-peak gradient of > 50 mm Hg is required. So we would have a patient with severe aortic stenosis who is restricted to most sports, but is not offered any corrective treatment. On the other hand, the same patient can receive two diametrically opposed recommendations according to the guidelines with which he is assessed, further reason for the need for an update of the American guidelines. Finally, neither of the two guidelines mention the participation of young children in competitive sport, a gap that needs to be addressed in future updates 

More than an update, what is needed is a harmonization of the recommendations issued by the different societies so that the message we transmit to our patients is consensual, clear and common. 

REFERENCE: 

Shibbani K, Abdulkarim A, Budts W, Roos-Hesselink J, Müller J, Shafer K et al. Participation in Competitive Sports by Patients With Congenital Heart Disease: AHA/ACC and EAPC/ESC/AEPC Guidelines Comparison. J Am Coll Cardiol. 2024 Feb 20;83(7):772-782. doi: 10.1016/j.jacc.2023.10.037.

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