Aerobic Exercise in Patients With Pulmonary Hypertension

This is an interventional supportive care trial for Pulmonary Hypertension focused on measuring Interstitial Lung Disease, Pulmonary Rehabilitation Read More

• INCLUSION CRITERIA:

PH and ILD-only Groups: Subjects of this study will include individuals with ILD and PH who are referred for Pulmonary Rehabilitation. Patients with ILD, PH or PH comorbid with ILD will be included. The following list provides more specific inclusion criteria:

• Between age 21-82 years
• WHO functional class II or III, will accept WHO functional class I and IV based on 6 minute walk test results (less than or equal to 400 meters for Class I and greater than or equal to 50 meters for Class IV)
• No recent syncope or significant chest pain
• No prior Pulmonary Rehabilitation received within the last 6 months.
• Physically inactive, no participation in a structured exercise program 3 or more days a week for over 30 minutes at each session including pulmonary rehabilitation maintenance within the last 6 months.

• Patients may qualify if they have any one of the following conditions:

  • PAH diagnosed by right heart catheterization defined as resting pulmonary mean arterial pressure equal to or higher than 25 mmHg

  • Interstitial lung disease, including idiopathic pulmonary fibrosis (IPF), non-specific pulmonary fibrosis (NSPF), sarcoidosis or other form of chronic lung fibrosis will be based on clinical context via clinic note from primary pulmonologist and an echo within one year of enrollment showing an RVSP <40mmHg.

    1. If RVSP is lower than 40mmHg patients with interstitial lung disease will be enrolled without the need for a right heart catheterization.
    2. If RVSP is indeterminate on an echo procedure performed within a year of enrollment, the patient will undergo another echo test at The National Institutes of Health, Echocardiogram Laboratory.
    3. Only if RVSP is unable to be estimated on an echo at NIH, the absence of the following abnormalities on echo will be used: right ventricular enlargement, right ventricular hypertrophy, paradoxical movement of interventricular septum and/or altered pulmonary flow velocity. If RVSP is unable to be estimated and these abnormalities are absent on echo, the patient will be enrolled as an ILD-only patient. If any of these echocardiographic abnormalities are present the patient will not be enrolled until the results of a right heart catheterization can be obtained to verify the absence of pulmonary hypertension.

Healthy Controls: The controls will include individuals who have never been diagnosed with primary or secondary PH and are apparently healthy. Controls will be matched to the PH cohort for age, gender, and body mass.

• Between 21 82 years
• Physically inactive, no participation in a structured exercise program 3 or more days a week for over 30 minutes at each session.
• No cardiorespiratory or pulmonary disease
• No other diseases of the neurological, metabolic, renal, or musculoskeletal system
• No medications that would influence aerobic capacity or treadmill performance

EXCLUSION CRITERIA FOR PATIENTS WITH PH, ILD-ONLY AND HEALTHY CONTROLS:

Since the goal is to examine exercise responses and adaptations as affected by PH, patients will have no other (except the primary disease in those with secondary PH) medical conditions that would impair aerobic capacity or the ability to engage in physical activity. These conditions would include any of those affecting the cardiovascular, pulmonary, metabolic, neurological, or musculoskeletal systems. Specific exclusion criteria for the PH and control cohorts are:

• Significant Restrictive or Obstructive Lung Disease with a FEV1/FVC ratio less than or equal to 65 percent
• Diagnosis of ischemic heart disease
• Left ventricular dysfunction with the ejection fraction less than 40 or a documented pulmonary capillary wedge pressure greater than or equal to 18 mmHg.
• Acute cor pulmonale
• Dilated or hypertrophic cardiomyopathy
• Non-idiopathic cardiomyopathy
• Significant hepatic or renal dysfunction
• Metastatic cancer with a life expectance of less than 6 months
• Disabling stroke
• Active substance abuse
• Severe psychiatric disease
• Patients on Antiretroviral Therapy
• Uncontrolled diabetes mellitus with a history of DKA
• Mitochondrial disease
• Pregnancy
• Ongoing tobacco use
• Children: Individuals younger than 21 years will not be included in the protocol because reference ranges for normative aerobic capacity and aerobic fitness have not been established for these age ranges.