Respiratory and Physical Therapy in Patients With Associated Pulmonary Arterial Hypertension (APAH) With Congenital Heart Defects

Study Overview
=================
Brief Summary
-----------------
The purpose of this study is to investigate the influence of physical training on exercise capacity, quality of life, functional class, oxygen consumption and right ventricular function in patients with severe associated pulmonary arterial hypertension (APAH) as part of a congenital heart defect with / without Eisenmenger's Syndrome

Official Title
-----------------
Influence of Respiratory and Exercise Therapy on Oxygen Uptake, Quality of Life in Patients With Severe Associated Pulmonary Arterial Hypertension (APAH) as Part of a Congenital Heart Defect With / Without Eisenmenger's Syndrome

Conditions
-----------------
Pulmonary Arterial Hypertension, Eisenmenger Syndrome

Intervention / Treatment
-----------------
* Other: respiratory and exercise therapy


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: signed consent form men and women> 18 years <80 years APAH with congenital heart defects with / without Eisenmenger syndrome (WHO functional class II-IV), invasively diagnosed by right heart and left heart catheterization: mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg, with targeted PAH medication for at least two months stable before study inclusion (exception: compensated WHO class II without vasodilating drug therapy) Exclusion Criteria: Pregnancy or lactation Change in medication during the last 2 months severe walking disturbance uncertain diagnoses No previous invasively confirmation of PH acute diseases, infections, fever Serious lung disease with FEV1 <50% or TLC <70% of target Further exclusion criteria are the following diseases: active myocarditis, unstable angina pectoris, exercise-induced ventricular arrhythmias, recurrent syncope within 4 weeks before study entry

Ages Eligible for Study
-----------------
Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

Primary Purpose: Supportive Care
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Respiratory and exercise therapy<br>Randomized, prospective, controlled, blinded study of three-week inpatient rehabilitation and subsequent continuing of the training at home for 12 weeks. The control group received conventional rehabilitation without a specific training program. After 15 weeks training is also offered to patients in the control group. | Other: respiratory and exercise therapy<br>* Conventional therapy with specific respiratory and physical therapy plus mental walking training<br>|
| No Intervention: Control group without exercise training<br>patients of the control group continue their sedentary lifestyle without given advice for exercise training. The time before start of rehabilitation (three months) serves as control group. Afterwards patients take part in the training program as well. | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in the maximum 6-minute walk distance (6MGT) |  | up to 15 weeks | 
| Changes in quality of life |  | up to 15 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in hemodynamics | Changes in maximum oxygen uptake Changes in exercise capacity: 6-minute walk distance, Recumbent Bike (Watts), respiratory economy (EQO2, EQCO2) Improved condition(NYHA class, Borg scale) Changes in Magnetic resonance tomography and echocardiographic parameters of right and left ventricle: size and pump function. Change of laboratory parameters, which are markers of right heart failure as NTproBNP, interleukins | up to 15 weeks |