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

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

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

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