Overview
Children and adults with pulmonary arterial hypertension (PAH) have severely reduced daily activity compared to healthy populations. In adults, investigators recently demonstrated that lower baseline daily step counts associated with increased risk of hospitalization and worsening WHO functional class; similarly, reduced step counts associate with hospitalization in children with PAH. This application builds on our recently completed NIH-funded pilot mobile health (mHealth) trial in adult patients with PAH which demonstrated the ability to remotely increase step counts. The investigators now aim to: (1) adapt our mHealth intervention to the developmental needs and interests of adolescents; and, (2) determine if our intervention increases step counts in adolescents, providing the foundation for a larger trial to assess the impact on quality of life and clinical outcomes.
Eligibility
Inclusion Criteria:
- Adolescents between ages 13-19 years.
- Diagnosed with idiopathic, heritable, or associated (connective tissue disease, drugs, or toxins) pulmonary arterial hypertension (PAH), or PAH due to simple congenital heart disease (i.e. atrial septal defect).
- WHO functional class I-III
- Stable PAH-specific medication regimen for three months prior to enrollment. Subjects with only a single diuretic adjustment in the prior three months will be included. Adjustments in IV prostacyclin for side effect management are allowed.
- Forced vital capacity >65% predicted with no or minimal interstitial lung disease based on reviews of imaging studies by PI and medical monitor.
Exclusion Criteria:
- Prohibited from normal activity due to wheelchair bound status, bed bound status, reliance on a cane/walker, activity-limiting angina, activity-limiting osteoarthritis, or other condition that limits activity.
- Pregnancy
- Diagnosis of PAH etiology other than idiopathic, heritable, or associated.
- Functional class IV heart failure
- Requirement of > 2 diuretic adjustment in the prior three months.
- Preferred form of activity is not measured by an activity tracker (swimming, yoga, ice skating, stair master, or activities on wheels such as bicycling or rollerblading).
- Involved in any other investigational intervention.