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

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