CVD Risk Profile in Children With HCM
Condition: Hypertrophic Cardiomyopathy
MeSH terms: Cardiomyopathies; Cardiomyopathy, Hypertrophic; Hypertrophy
Study type: Observational

Summary: 
      Hypertrophic cardiomyopathy is a disease of the heart muscle that causes the heart to become
      thicker and this thickness places children at risk of heart rhythm problems, heart failure
      and sudden death.To decrease the risk of sudden death, health care providers generally
      counsel that the patient should stop all intense physical activity. While this recommendation
      may decrease the risk of sudden death it is unclear what the long term impact of reduced
      physical activity is on cardiovascular health in children with HCM.

      Cardiovascular (CV) disease is a disease of the heart and blood vessels and is the cause of
      heart attacks in adults. There are many risk factors for the development of CV disease
      including genetics, medical conditions and lifestyle choices. While some studies in adults
      suggest that patients with HCM are at higher risk of poor cardiovascular health, this has not
      yet been assessed in children. Although, CV disease is generally thought of to be a disease
      of adults, there is a lot of information that suggests the development of CV disease starts
      early in life and therefore by promoting heart healthy lifestyles in children, it is possible
      that these children will becomes healthier adults.

      The goal of this project is to assess risk factors for CV disease in a population of children
      with HCM at the two largest pediatric cardiac programs in Canada. This assessment will be to
      look at factors we can measure (e.g., weight, cholesterol levels) and patients' and families'
      perceptions of what it means to be heart healthy. It is hoped that through this project risk
      factors for heart disease, and poor "heart healthy" lifestyles choices, will be identified in
      order to develop strategies to decrease these risk factors in patients with HCM. With a
      better understanding of the families' perceptions of heart healthy behaviours, educational
      tools and resources for cardiovascular health promotion in patients with HCM can be
      developed.
    

Detailed description: 
      Hypertrophic cardiomyopathy (HCM) is an inherited cardiomyopathy with the hallmark feature of
      left ventricular hypertrophy primarily involving the ventricular septum. It is associated
      with an increased risk of arrhythmias and sudden cardiac death (SC) [Maron et al., 2016]. It
      is the most common genetic heart disease, with a prevalence of up to 1 in 200 in the general
      population.

      It is widely acknowledged that regular physical activity is important for long-term CV health
      in the general population. Childhood obesity is on the rise in the general population and
      physicians may be compounding the risk in children with HCM due to the activity restrictions
      imposed on them. Given the significant emphasis placed on what physical activities patients
      with HCM should avoid, physicians may not be effectively communicating the importance of
      maintaining a heart healthy lifestyle, including the importance of regular physical activity,
      on long-term CV health. This theory could explain the high levels of inactivity and obesity
      seen in the adult population of individuals with HCM, which have important consequences on
      long-term health and well-being.

      Little research has been done to examine the other markers of CV health in the adult
      population. In addition, there have not been any studies evaluating the CV disease risk
      profile of children and adolescents with HCM and whether the phenomenon seen in the adult
      population is also seen in pediatric patients. This study will fill an important gap which
      will allow for the development of safe interventions in this unique patient population
      focused on promoting life-long CV health.

      The primary aims of the study are:

        1. To describe the CV disease risk behaviors and CV disease risk profile of children with
           HCM.

        2. To determine if children with HCM are at greater risk for acquired CV disease compared
           to the general population.

      The secondary aim of the study are:

        1. To identify barriers to a heart healthy lifestyle.

        2. To identify both the children's and families' perception of a heart healthy lifestyle.

      This study will employ multiple methods. The quantitative component will be a prospective
      cross-sectional study undertaken at two centers in Canada with a dedicated Heart Function and
      Cardiomyopathy Department. The quantitative component will be a prospective cross-sectional
      study undertaken at across pediatric cardiac centers in Canada who look after children with
      HCM.
    

Observational model: Cohort
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        Children 10 - 19 years of age with primary hypertrophic cardiomyopathy. We are choosing to
        only include children with HCM as they often are told to not participate in strenuous
        activities b/c of the risk of sudden death. This patient population also has not been well
        studied and they often have other co-morbidities that may prevent exercise