Please select the eligibility criteria that correspond to the clinical trial below.
Title: N/A
Brief Summary: Patients suffering from pulmonary arterial hypertension (PAH) frequently remain symptomatic despite medical therapy. Symptoms include breathlessness, poor exercise capacity and reduced quality of life.

In many other serious heart or lung diseases it has been shown that physical rehabilitation improves patient's fitness and quality of life. In PAH there are no clear guidelines and in general physical activity has traditionally been discouraged, although evidence for this advice is lacking. Interesting research project in Germany showed significant benefit for in-patient rehabilitation in PAH patients.

In this study we will perform a controlled clinical study of out-patient rehabilitation of patients with PAH. We hypothesize that physical training of patients will result in increased exercise capacity and improved quality of life.


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:

* Clinically stable
* Under standard medication
* Group 1 pulmonary arterial hypertension patients
* Having no exacerbation or infection

Exclusion Criteria:

* Cognitive disorders
* Orthopedic and neurological problems
* Pneumonia or acute infection
* Stage 4 heart failure
* Having no comorbidity to avoid performing evaluation and training
C: 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
D: Inclusion Criteria:

* All patients must satisfy current diagnostic criteria for pulmonary artery hypertension based on their historical right heart catheter data (within 4 years of study enrollment): Mean PAP \>25mmHg at rest or \>30mmHg with exercise, by a PCWP \<= 15mmHg and by PVR \>3 Wood Units.
* Willing and able to participate in 24 bi-weekly rehabilitation sessions, and medical follow-up.
* Stable dose of current PAH-specific medication for 3 months prior to enrollment.
* New York Heart Association (NYHA) Class II-III.
* Women of child-bearing age must demonstrate adequate contraception or undergo a pregnancy test.

Exclusion Criteria:

* Functional Class NYHA Class I or IV.
* PAH due to congenital heart disease, left heart disease, chronic lung diseases (VC or FEV1 \< 60% of predicted) or chronic hypoxia.
* Acute intercurrent illness requiring hospital admission in the month proceeding screening.
* Any non-PAH medical condition likely to interfere with participation in rehabilitation, e.g. musculoskeletal disorders.
* Any uncontrolled or terminal non-PAH medical condition likely to interfere with completion of the study, according to the judgment of the study physician.
* Participation in another rehabilitation scheme within 6 months of enrollment in the study.
* Current participation in another clinical trial.
* Pregnancy or planned pregnancy during the study period.

Answer: D