Cardiopulmonary Fitness and Physical Activity Among Children and Adolescents With Inherited Cardiac Disease
Authors: Souilla Luc , Werner Oscar , Huguet Helena , Gavotto Arthur , Vincenti Marie , Pasquie Jean-Luc , De La Villeon Gregoire , Guillaumont Sophie , Pommier Victor , Matecki Stefan , Baruteau Alban-Elouen , Beyler Constance , Delclaux Christophe , Denjoy Isabelle , Charron Philippe , Chevalier Philippe , Delinière Antoine , Andrianoely Mathieu , Cornuault Lauriane , Besnard-Neyraud Caroline , Sacher Frederic , Reant Patricia , Mottet Denis , Picot Marie-Christine , Amedro Pascal ,   Quality of Life in Children With Inherited Cardiomyopathy or Arrhythmia (QUALIMYORYTHM) Study Group, Abassi Hamouda , Boisson Aymeric , Requirand Anne , Auer Annie , Probst Vincent , Prigent Solène , Hauet Quentin , Haddad Christelle , Bertail-Galoin Claire , Iriart Xavier , Thomas-Chabaneix Julie , Duboue Pierre-Marie , Ruissel Amandine , Selly Jean-Bernard 
Published: February 2025

ImportanceHistorical restrictions on children with inherited cardiac arrhythmia or cardiomyopathy have been implemented to mitigate the potential risk of sudden death, but these limitations can be detrimental to overall health and cardiopulmonary fitness.ObjectivesTo evaluate cardiopulmonary fitness and physical activity among children with inherited cardiac disease and identify the factors associated with maximum oxygen uptake (V̇o2max) in this population.Design, Setting, and ParticipantsThis cross-sectional, multicenter, prospective controlled study was conducted in 7 tertiary care expert centers for inherited cardiac disease in France from February 1, 2021, to June 20, 2023, with a 2-week follow-up. Participants included 100 children and adolescents aged 6 to 17 years with inherited cardiac arrhythmia or cardiomyopathy who were compared with 107 sex- and age-matched controls.Main Outcomes and MeasuresMaximum oxygen uptake was assessed using cardiopulmonary exercise testing, and results were expressed using pediatric reference z score values. The main determinants of V̇o2max included clinical (New York Heart Association [NYHA] functional class, treatment, echocardiographic, and electrocardiogram variables), functional (cardiopulmonary exercise test parameters), sociodemographic (sex, schooling, and parents’ education), and behavioral (physical activity and motivation) characteristics.ResultsA total of 100 patients (mean [SD] age, 12.7 [3.1] years; 52 boys [52.0%]) and 107 controls (mean [SD] age, 11.7 [3.3] years; 54 boys [50.5%]) were included. The V̇o2max was lower in patients than controls, expressed as z scores (mean [SD] score, −1.49 [1.48] vs −0.16 [0.97]; P &amp;lt; .001) or raw values (mean [SD] value, 32.2 [7.9] vs 40.2 [8.5] mL/kg/min; P &amp;lt; .001). Moderate to vigorous physical activity levels were lower in patients than in controls (mean [SD] level, 42.0 [23.6] vs 48.2 [20.4] min/d; P = .009). The final multivariable model explained 80% of the V̇o2max by integrating clinical (lower NYHA functional class, absence of ventricular dilatation, and absence of implantable cardioverter-defibrillator), functional (higher forced vital capacity and ventilatory anaerobic threshold), sociodemographic (male sex, normal progression of schooling, and higher maternal educational level), and behavioral (higher self-reported physical activity and motivation toward physical activity) parameters.Conclusions and RelevanceThis cross-sectional study suggests that levels of cardiopulmonary fitness and physical activity were lower in children and adolescents with inherited cardiac disease than in healthy controls, even after adjusting for use of β-blockers and using modern pediatric reference models. Assessing cardiopulmonary fitness among children with inherited cardiac disease can contribute to engaging in a shared decision-making process for sports participation and preventive interventions, such as early cardiac rehabilitation programs.