Rehabilitation for Patients With Pulmonary Hypertension

The purpose of this study is to test the impact of a personalized, partly supervised rehabilitation program on the exercise capacity in patients with pulmonary hypertension. The rehabilitation program consists of 2 weeks inpatient, 2 weeks ambulatory and 11 weeks home based rehabilitation.

The present study is a 15 weeks, randomized (1:1) controlled trial. Patients in both groups will not change their usual medical treatment. After 2 clinical visits (screening and randomization visit), patients in the intervention group will follow a 15 week rehabilitation program consisting of 2 weeks inpatient, 2 weeks outpatient and 11 weeks home based rehabilitation. At the end of the outpatient rehabilitation phase (week 4) and the home-based program (week 15), clinical outcomes will be reassessed.

Eligibility Criteria:
Inclusion Criteria:

* Female and male patients ≥ 18 years
* Pulmonary arterial hypertension (PAH, Group 1 of Nice classification) and chronic thromboembolic pulmonary hypertension (CTEPH, group 4)
* WHO functional class II-III
* PH diagnosed by right heart catheter showing:

  1. Baseline mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg
  2. Baseline pulmonary vascular resistance (PVR) ≥ 240dyn x s x cm-5
  3. Baseline pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHgg
* Patients receiving optimized conventional PH therapy including intensified treatment with diuretics and who have been stable for 2 months before entering the study.
* Except for diuretics, medical treatment should not be expected to change during the entire 15-week study period.
* Negative pregnancy test (β-HCG) at the start of the trial and appropriate contraception throughout the study for women with child- bearing potential
* Able to understand and willing to sign the Informed Consent Form

Exclusion Criteria:

* PH of any cause other than permitted in the entry criteria, e.g. concomitantly to portal hypertension, complex congenital heart disease, reversed shunt, HIV infection, suspected pulmonary veno-occlusive disease based on pulmonary edema during a previous vasoreactivity test or on abnormal findings compatible with this diagnosis (septal lines or pulmonary edema at high resolution computer tomography), congenital or acquired valvular defects with clinically relevant myocardial function disorders not related to pulmonary hypertension or unclear diagnosis
* Patients with signs of right heart decompensation
* Walking disability
* Acute infection, pyrexia
* Any change in disease-targeted therapy within the last 2 months
* Any subject who is scheduled to receive an investigational drug during the course of this study
* Severe lung disease: FEV1/FVC \<0.5 and total lung capacity \<70% of the normal value
* Active myocarditis, instable angina pectoris, exercise induced ventricular arrhythmias, decompensated heart failure, active liver disease, porphyria or elevations of serum transaminases \>3 x ULN (upper limit of normal) or bilirubin \>1.5 x ULN
* Hemoglobin concentration of less than 75% of the lower limit of normal
* Systolic blood pressure \<85 mmHg
* History or suspicion of inability to cooperate adequately