Please select the eligibility criteria that correspond to the clinical trial below.
Title: Response and Adaptation to Aerobic Exercise in Patients With Pulmonary Hypertension: Initial Studies for Establishing Guidelines
Brief Summary: This study will determine if a rehabilitation exercise program can help people with pulmonary hypertension (PH) increase their physical activity. Patients with PH have an increase in blood pressure in the pulmonary blood vessels (artery, vein or capillaries) that leads to shortness of breath, dizziness, fainting and other symptoms.

Healthy volunteers and people with pulmonary hypertension between 21 and 75 years of age may be eligible for this study.

All participants undergo the following tests and procedures:

* Medical history and physical examination
* 6-minute walk test: Subjects walk as fast as they can for 6 minutes on a walking track to determine their ability to participate in physical activity.
* Questionnaires: Subjects complete nine questionnaires related to their fatigue, daily physical activity, mood, and so forth.
* Maximum treadmill test: The exercise begins at an easy level and gradually increases until the subject says he or she can no longer continue or the investigator decides it is not safe to continue. Subjects are fitted with a mask, electrodes and light sensors to measure how well the heart is working and how well the muscles use oxygen.

Patients with pulmonary hypertension undergo the following additional procedures:

* Activity monitoring: Patients wear a monitor for 3 days that measures movement and heart rate.
* Group assignment: Patients are randomly assigned to Group 1 (education plus aerobic exercise) or Group 2 (education followed by exercise).
* Group 1 patients will attend classes three days a week at either Inova Fairfax Hospital Pulmonary Rehabilitation Center or The National Institutes of Health for 10 weeks. Two sessions a week will include a 1 hour education session as well as a 30-45 minute track or treadmill exercise session. The third session will only include exercise. During the education patients will learn about a healthy lifestyle with pulmonary hypertension. After the 10 weeks of education and exercise, subjects repeat the 6-minute walk test, maximum treadmill test and questionnaires.
* Group 2 patients participate in 2; 1-hour educational session at either the Inova Pulmonary Rehabilitation Center or The National Institutes of Health for 10 weeks. After the classes, they repeat the 6-minute walk test, maximum treadmill test and questionnaires. The following 10 weeks will consist of 3 days a week of 30-45 minute track or treadmill walking at either Inova or NIH, after which they again repeat the questionnaires, treadmill and walk tests.


Options:
A: * 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.
B: Inclusion Criteria:

* age \>18 years
* informed consent signed by patient to conduct the study
* no diseases excluding rehabilitation
* pts with idiopathic, inherited PAH (pulmonary arterial hypertension), or in the course of connective tissue diseases, portal hypertension, congenital heart diseases.
* PAH confirmed in catheterisation of right side of heart mPAP (mean pulmonary arterial pressure) ≥25 mmHg, PCWP (pulmonary capillary wedge pressure) ≤15mmHg.
* Chronic heart failure - left ventricle ejection fraction \<40% in echocardiography, NYHA \*New York Health Association) class II-III
* stable ischaemic heart disease in CCS (Canadian Cardiovascular Society) class II-III

Exclusion Criteria:

* Other types of pulmonary hypertension
* COPD (chronic obstructive pulmonary disease), asthma
* malignancies
* Acute inflammatory state up to 4 weeks before inclusion to the study
* Acute coronary syndrome up to 3 months before inclusion to the study
* Heart failure in NYHA IV class
* severe anaemia (Hgb \<11g/dl for men \<10g/dl for women)
* electrolyte and hormonal disturbances in period of 1 month before inclusion to the study
* substantial modification of treatment of main disease within last 3 months
* ischaemic heart disease in CCS class IV
* Other clinical situations excluding to perform controlled program of rehabilitation
C: Inclusion Criteria:

* Patients with severe chronic PH who were stable and compensated under optimized medical therapy (such as endothelin antagonists, iloprost, sildenafil, calcium channel blockers, anticoagulants, diuretics, and supplemental oxygen) for at least 3 months before entering the study will be invited to participate.

Additional inclusion criteria will be age of between 18 and 75 years and World Health Organization (WHO) functional class II to IV.

Exclusion Criteria:

* Patients with recent syncope or skeletal or muscle abnormalities prohibiting participation in an exercise program won't be included.
D: Inclusion Criteria:

* Men and women age 18-90 years
* Signed informed consent
* Symptomatic PAH (idiopathic, hereditary or associated with systemic scleroderma)
* Invasively confirmed PAH
* 6MWT ≥ 50 m
* Stable specific therapy for at least 2 months
* Ability to perform bike test
* Ability to perform wbv training
* NYHA/WHO-FC II-III

Exclusion Criteria:

* any other PAH/PH than idiopathic, hereditary or associated with systemic scleroderma
* Rehabilitation or other training concept performed within 2 months before inclusion
* pregnancy
* acute thrombosis
* newly implanted Hip or Knee
* recent bone fracture
* Disability to confirm consent
* NYHA/WHO-FC IV

Answer: A