Frailty is a complex biologic syndrome of diminished physiologic reserve that leads to decreased resistance to stressors and is associated with adverse health outcomes. The syndrome has been well studied in adults and is quantified by the Fried criteria, which are comprised of five components- slowness, weakness, self-reported exhaustion, shrinkage and diminished physical activity. The concept of frailty is novel in children. A study in young adult childhood cancer survivors demonstrated increased incidence of frailty in this population along with increased risk of morbidity and mortality. This suggests that frailty as a phenotype has relevance outside of the geriatric age group. Pediatric patients with single ventricle physiology, heart failure and pulmonary artery hypertension- all represent populations with significantly increased risk of mortality, morbidity and decreased quality of life. Currently, such patients are monitored outpatient by serial echocardiograms and blood work that only gives information about end organ damage. But there is no validated tool available to measure global infirmity in such children. Better understanding of the relevance and applicability of frailty in pediatrics may allow for identification of the most vulnerable pediatric cardiac patients and be of value in optimizing their clinical management and improving health outcomes.

Inclusion Criteria:

SUBJECT/CASE POPULATION herein referred to as Subjects:

• Children and adolescents between the ages of 8.0-17.50 years

• Subjects must be diagnosed with one of the following heart conditions:

  • Subjects with single ventricle physiology and Fontan surgery completion at least 6 months prior to study enrollment
  • Subjects with diagnosis of heart failure by a cardiologist
  • Subjects with a diagnosis of pulmonary arterial hypertension, confirmed by cardiac catheterization, requiring use of at least 1 pulmonary vasodilator or oxygen for their pulmonary hypertension

CONTROL POPULATION herein referred to as Controls:

• Healthy children and adolescents between the ages of 8.0-17.50 years

Exclusion Criteria:

BOTH SUBJECT/CASE POPULATION AND CONTROL POPULATION:

• Subjects or controls with known severe neurological or respiratory diseases, eating disorders (such as anorexia, bulimia) or physical limitations (wheelchair bound) which may impact their ability to perform study procedures in the opinion of the provider.
• Subjects or controls with tracheostomy and ventilator dependency
• Subjects or controls with unstable angina/ myocardial infarction in the last 4 weeks
• Subjects or controls who are unable to perform 6 continuous minutes of walking, hand-grip dynamometry, or complete questionnaire measures as described.

SUBJECT/ CASE POPULATION:

• Subjects with heart transplantation within the past 1 year
• Subject will be excluded if the cardiologist feels that the study is not appropriate for the subject.

CONTROL POPULATION:

• Controls with any known chronic medical condition requiring some medication over the past 30 days.
• Controls on any chronic prescription medicines for > 30 days

The investigators are studying children with single ventricle physiology, s/p Fontan, patients with heart failure and pulmonary hypertension.