Review
Risks and Benefits of Exercise Training in Adults With Congenital Heart Disease

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Abstract

Exercise capacity in adults with various forms of congenital heart disease is substantially lower than that of the general population. Although the underlying congenital heart defect, and its sequelae, certainly contribute to observed exercise limitations, there is evidence suggesting that deconditioning and a sedentary lifestyle are important implicated factors. The prevalence of acquired cardiovascular comorbidities is on the increase in the aging population with congenital heart disease, such that obesity and a sedentary lifestyle confer increased risk. Health fears and misconceptions are common barriers to regular physical activity in adults with congenital heart disease, despite evidence linking lower functional capacity to poor outcomes, and data supporting the safety and efficacy of exercise in bestowing numerous physical and psychosocial rewards. With few exceptions, adults with congenital heart disease should be counselled to exercise regularly. In this contemporary review, we provide a practical approach to assessing adults with congenital heart disease before exercise training. We examine available evidence supporting the safety and benefits of exercise training. Risks associated with exercise training in adults with congenital heart disease are discussed, particularly with regard to sudden cardiac death. Finally, recommendations for exercise training are provided, with consideration for the type of congenital heart disease, the nature (ie, static vs dynamic) and intensity (ie, low, medium, high) of the physical activity, and associated factors such as systemic ventricular dysfunction and residual defects. Further research is required to determine optimal exercise regimens and to identify effective strategies to implement exercise training as a key determinant of healthy living.

Résumé

La capacité à l’effort des adultes souffrant de différentes formes de cardiopathies congénitales est considérablement plus faible que celle de la population générale. Bien que les anomalies cardiaques congénitales sous-jacentes et leurs séquelles contribuent sans aucun doute à l’observation de limites en matière d’exercice, des données scientifiques suggèrent que le déconditionnement physique et le mode de vie sédentaire sont d’importants facteurs associés. La prévalence des comorbidités cardiovasculaires acquises est en hausse dans la population vieillissante souffrant de cardiopathies congénitales, de sorte que l’obésité et les modes de vie sédentaires confèrent un risque accru. Les craintes et les idées fausses en matière de santé sont des obstacles communs à la pratique régulière de l’activité physique chez les adultes souffrant de cardiopathies congénitales, en dépit des données scientifiques reliant la capacité fonctionnelle plus faible à des résultats médiocres, et des données appuyant l’innocuité et l’efficacité de l’exercice en lui attribuant de nombreux avantages physiques et psychosociaux. À quelques exceptions près, les adultes souffrant de cardiopathies congénitales devraient être invités à faire régulièrement de l’exercice. Dans la présente revue contemporaine, nous proposons une approche pratique pour évaluer les adultes souffrant de cardiopathies congénitales avant l’entraînement à l’effort. Nous examinons les données scientifiques disponibles appuyant l’innocuité et les avantages de l’entraînement à l’effort. Nous discutons des risques associés à l’entraînement à l’effort chez les adultes souffrant de cardiopathies congénitales, particulièrement de la mort cardiaque subite. Finalement, nous donnons des recommandations en matière d’entraînement à l’effort en tenant compte du type de cardiopathie congénitale, de la nature (c.-à-d. statique vs dynamique) et de l’intensité (c.-à-d. faible, moyenne, élevée) de l’activité physique, et des facteurs associés comme la dysfonction du ventricule systémique et les anomalies résiduelles. Des recherches plus approfondies sont nécessaires pour déterminer les programmes d’exercices optimaux et établir des stratégies efficaces de mise en œuvre de l’entraînement à l’effort comme facteur déterminant d’un mode de vie sain.

Section snippets

Assessment Before Exercise Training in CHD

As outlined in Table 1, a thorough assessment is required before recommending exercise training in patients with CHD, with the purpose of informing the counselling process, identifying those at high risk for exercise-induced adverse events, and providing evidence to reassure patients concerning safety.8 One study reported that education of adults with CHD regarding exercise is suboptimal, even in a specialist clinic, leading to frequent misconceptions about potential benefits and dangers.9 A

Benefits of Exercise Training in CHD

The overarching objectives of exercise training in patients with CHD are to improve health, exercise tolerance, and quality of life. Several studies in diverse populations of patients with surgically repaired CHD have reported improvements in exercise tolerance after various exercise training programs, including VO2 max, peak oxygen consumption, peak oxygen pulse, total treadmill walking time, heart rate recovery, walking distance, power output, and oxygenation of respiratory muscles.11, 12, 13

Fontan Circulation

In complex patients with a Fontan circulation, exercise intolerance is multifactorial. In addition to the absence of a subpulmonary pump that contributes to a blunted increase in stroke volume with exercise, chronotropic incompetence and underlying arrhythmias might further impair exercise capacity. Although patients with a Fontan circulation often report the absence of symptoms during activities of daily living, objective functional capacity is characteristically reduced. The initial increase

Systemic Morphologic Right Ventricle

In patients with a systemic morphologic right ventricle due to congenitally or surgically (D-TGA) corrected transposition of the great arteries with atrial redirection, exercise capacity is limited by the inability to increase cardiac output as a result of chronotropic incompetence,24 inadequate ventricular filling due to poor atrial transport (ie, with Mustard or Senning baffles),25 impaired function of the systemic right ventricle,26 and/or limited coronary flow reserve.27, 28, 29, 30, 31 In

Other

Improvements in aerobic capacity as a result of structured exercise training have also been reported in small series of adults with tetralogy of Fallot16 and pulmonary hypertension associated with CHD.33 Other benefits of exercise training include the prevention of acquired coronary artery disease for which patients with CHD are becoming increasingly susceptible as a result of their improved longevity. There is evidence, albeit inconsistent, to suggest that obesity is more prevalent in patients

Risks Associated With Exercise Training in CHD

Feared complications associated with exercise in patients with CHD include malignant arrhythmias, exercise-induced hypertension, aortic dissection, myocardial ischemia, heart failure exacerbation, worsening cyanosis, syncope, and sudden cardiac death.39 All elements of the clinical profile should be considered in determining risks. For example, high-impact sports might be associated with additional risks in patients receiving anticoagulation therapy or with implantable

Recommendations for Exercise Training in CHD

The Canadian Cardiovascular Society has not issued specific recommendations regarding exercise training in patients with CHD. In 2008, the American College of Cardiology/American Heart Association (AHA) management guidelines for adults with CHD recognized the importance of individualizing, and regularly updating, exercise prescription.39 The American College of Cardiology/AHA guidelines endorsed published recommendations for exercise defined by the 36th Bethesda conference on CHD.50 In

Conclusions

A wealth of observational literature supports a strong association between higher levels of physical activity and lower rates of obesity, hypertension, and cardiovascular disease, superior psychosocial outcomes, and improved survival. Although the literature regarding exercise in CHD is limited to a few studies on relatively small patient populations with short-term follow-up, results are consistent in showing that the anticipated benefits of exercise are applicable to the growing population

Funding Sources

Dr Khairy is supported by a Canada Research Chair in Adult Congenital Heart Disease and Electrophysiology.

Disclosures

The authors have no conflicts of interest to disclose.

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