Healthy-related Quality of Life and Physical Activity of Children With Cardiac Malformations

Study Overview
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Brief Summary
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The QUALIMYORYTHM trial is a multicentre controlled study, aiming to assess health-related quality of life (HRQoL) of 107 children aged 6 to 18 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathies (hypertrophic, dilated, or restrictive cardiomyopathy), and to compare the results to those of 107 age and gender-matched healthy subjects. The secondary objective is to assess, in this population, the HRQoL according to disease characteristics, level of physical activity, exercise capacity, and socio-demographic data. Participants will wear a fitness tracker for 2 weeks.

Detailed Description
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Advances in paediatric cardiology has improved the prognosis of children with inherited cardiac disorders. However, quality of life and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathies. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial. The QUALIMYORYTHM trial is a multicentre controlled study. The main objective is to compare health-related quality of life (HRQoL) of children aged 6 to 18 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathies (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess, in this population, HRQoL according to the disease clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity (VO2max), and the socio-demographic data. Participants will wear a fitness tracker (actimeter watch) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population.

Official Title
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Healthy-related Quality of Life and Physical Activity of Children With Inherited Cardiac Arrhythmia or Inherited Cardiomyopathies: the Prospective Multicentre Controlled QUALIMYORYTHM Study

Conditions
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Long QT Syndrome, Brugada Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, Arrhythmogenic Right Ventricular Dysplasia, Hypertrophic Cardiomyopathy, Dilated Cardiomyopathy, Restrictive Cardiomyopathy

Intervention / Treatment
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* Other: MRI


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Male or female aged 6 to 18 years old. Group 1: Patients with an inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or those with an inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy). Group 2: Children with a completely normal check-up, referred to the paediatric cardiology consultation for a non-severe functional symptom linked to exercise (murmur, palpitation, or dyspnoea) or for a medical sports certificate. Informed consent of parents or legal guardians, and oral assent of children Exclusion Criteria: Patients who are not able to understand or fill out the questionnaires (QoL, physical activity and motivation questionnaires). Absolute contraindications for CPET: fever, uncontrolled asthma, respiratory failure, acute myocarditis or pericarditis, uncontrolled arrhythmias causing symptoms or haemodynamic compromise, uncontrolled heart failure, acute pulmonary embolus or pulmonary infarction, and children with mental impairment leading to inability to cooperate. Group 2: Children with any chronic disease, medical condition (cardiac, neurologic, respiratory, muscular, or renal), or medical treatment and those requiring any further specialized medical consultation.

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Cardiac disease<br>Children aged of 6 to 18 years old with an inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or those with an inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy). | Other: MRI<br>* MRI cardiac<br>|
| Sham Comparator: Control group<br>Children aged 6 to 18 years old referred to the paediatric cardiology consultation who were classified in the control group after a completely normal check-up, including physical examination, electrocardiogram, and echocardiography. | Other: MRI<br>* MRI cardiac<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Total score of health-reported quality of life self questionnaire | PedsQL questionnaire | baseline (1 day) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The metabolic equivalent of task | measured by the wearable actimeter device. | during 14 days after inclusion | 
| physical activity level | Ricci and Gagnon questionnaire | baseline (1 day) | 
| Motivation towards health-oriented physical activity | EMAPS motivation scale | baseline (1 day) | 
| Exercise capacity | Cardiopulmonary exercise test with VO2max assessment | baseline (1 day) | 
| Myocardial morphologic assessment | cardiac MRI | baseline (1 day) | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Life quality, Physical activity, Paediatric cardiology, Inherited cardiac arrhythmia, Genetic cardiomyopathies