Physical Activity Promotion in Children and Adolescents With Single Ventricle Physiology (MedBike)
Condition: Congenital Heart Defect; Hypoplastic Left Heart Syndrome; Physical Activity
MeSH terms: Heart Defects, Congenital; Univentricular Heart; Hypoplastic Left Heart Syndrome
Study type: Interventional

Summary: 
      Single ventricular (SV) heart was a uniformly fatal condition before the advances in surgical
      treatment in 1980. In the present era, 5-year survival rate with SV is 75%, with some
      centers, including the Stollery Children's Hospital reporting higher survival. Although
      mortality remains a major concern, the research focus has shifted to management of late
      complications as well as improving patient physical and mental health related quality of life
      issues. Children with SV have reduced exercise tolerance and this is progressive through
      adulthood. Recent advances in remote health assessment and telehealth systems have allowed
      the development of medically supervised home graduated physical training for adult cardiac
      patient rehabilitation. To our knowledge, the application of these technologies has not been
      applied to SV patients. The long term goal is to use this technology to improve patient
      exercise capacity and to positively influence parental and patient perceptions of the
      patient's physical ability.
    

Detailed description: 
      Children with single ventricular (SV) heart were a uniformly fatal condition prior to
      descriptions of surgical palliation in 1980 that results in a Fontan circulation with single
      ventricle physiology. In the present era, 5-year survival is 75% in multicenter studies, with
      some centers including the Stollery Children's Hospital reporting higher survival. Although
      mortality remains a challenge for this population, the research focus has shifted towards
      optimizing management of late complications as well as improving patient physical and mental
      health-related quality of life issues. Children with SV have decreased exercise tolerance and
      the reduction is progressive through adolescence and into adulthood. Physical factors
      contributing to decreased exercise tolerance in the Fontan patient include cardiovascular
      inefficiencies and the loss of peripheral lean muscle mass and efficiency from
      deconditioning. Recently, Cordina et al. has shown that an intensive exercise regiment can
      improve muscle strength and mass, cardiac output and exercise capacity in adults with Fontan
      circulation. The majority of published outcomes from exercise training in patients with
      congenital heart disease (CHD) have resulted in increased measured exercise capacity with no
      apparent negative effect. Although the link between improved exercise capacity and improved
      patient quality of life remains controversial, participation in an aerobic exercise regimen
      leads to improved health-related quality of life.

      Aside from physical limitations to their exercise capacity, Fontan patient self-confidence
      toward physical activity is low, as is exercise participation. Studies indicate that amongst
      youth with CHD, low self-confidence may be a more important predictor of participation than
      the severity of the disease. Parental overprotection is a common finding in children with CHD
      with a lasting impact on patient self-confidence and anxieties toward physical activity well
      into adulthood. Alteration of patient and parental perception and anxieties toward
      participation in physical activity may improve compliance to exercise training and encourage
      more positive patient perceptions toward healthy lifestyle habits, including frequent
      physical activity participation.

      Recent technological advances in remote health assessment capabilities and telehealth systems
      have allowed the development of medically supervised home graduated physical training for
      adult cardiac patient rehabilitation. The application of such technologies to pediatric
      congenital heart patients has not been tested. In collaboration with Prof. Boulanger at the
      Advanced Man Machine Interface Laboratory at the University of Alberta, a custom pediatric
      remote bike ergometer (MedBike) was developed. This technology provides the medical
      supervisor with a live-feed of patient video/audio, electrocardiograph (ECG), blood pressure
      (BP) and blood oximetry signals while enabling remote determination of patient work load
      through the bike ergometer. The long-term goal is to use this technology to improve patient
      exercise capacity and to positively influence patient and parental perceptions of the
      patient's physical ability.

      Stage 2 (HIIT exercise program): The investigators will evaluate the safety and efficacy of
      an 8-week, 3 times per week supervised HIIT exercise program in patients with SV physiology.

      All eligible patients will undergo a full cardio pulmonary exercise testing (CPET) and
      anthropometry assessment of lean muscle mass prior to exercise training and at the end of the
      training period. Understanding that a patient's CPET results may change over time, subjects
      who participated in Stage 1 of the study will be invited back to undergo a new baseline CPET
      to ensure the reliability of the data.

      A MedBike will be installed in the participant's home. Members of the MedBike team at the
      University of Alberta will be responsible for installation, set-up, and training with regards
      to participant use of the MedBike. Installation, set-up, and training will occur at a time
      that is convenient for the participant and their caregivers. The patients will exercise in
      the convenience of their home with tele-health link that includes a live video and audio feed
      to the supervisor workstation at the University of Alberta. The HIIT program described above
      will be applied. The supervisor will have the ability to modify the program intensity during
      each session based on the perceived difficulty or ease of it and the results of the baseline
      CPET. The exercise sessions in their home will be also be supervised in-person by the
      patient's caregiver. Given that no adverse events or safety concerns arose in Stage 1B of the
      study, the patient will be supervised remotely by a member of the MedBike team capable of
      reading ECG data. The investigators will document any adverse effects from the 240 training
      sessions during the study.

      Exercise sessions will be based on standardized guidelines for aerobic exercise (ACSM's
      guidelines for exercise testing and prescription 2013). Heart rate, ECG, oxygen saturation
      and rating of perceived exertion will be monitored during each session.

      Any adverse events such as profound desaturation (oxygen saturations fall of > 10% points for
      greater than 1 min, chest pain and ECG changes consistent with ischemia (ST depression or
      elevation in 2 consecutive leads), development of tachyarrhythmia (atrial or ventricular) and
      any bike injury, will result in immediate stoppage of exercise regimen and evaluation by the
      remote supervisor with access to medical personnel..

      The impact of the exercise program on patient and parental perceptions of the patient's
      physical capacity will be evaluated using qualitative methods, as well as health related
      quality of life questionnaires. Patient and parents will be interviewed prior to, and at the
      end of, the exercise training as to their perceptions of the influence of physical activity
      in the presence of complex CHD.
    

Intervention name(s): MedBIKE
Intervention type(s): Device
Intervention description(s): A MedBIKE will be installed into a participants home and connected to clinicians via telehealth for monitoring during exercise sessions. Sessions will be based on standardized guidelines for aerobic exercise. Heart rate, ECG, oxygen saturation and rating of perceived exertion will be monitored throughout the session.
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Other