Increased pulmonary precapillary vascular resistance due to vasoconstriction and vasoproliferative processes is the basic pathophysiological mechanism in the development of pulmonary hypertension (PH). In patients with pulmonary arterial hypertension (PH) production of endothelin-1 (ET-1) is increased and elevated ET-1 plasma levels correlate with PH severity As recently shown Autoantibodies against the Endothelin-1 Typ A and Angiotensin II Typ-1 Receptor, which have a high Incidence in PH-Patients, may also play an important role in the pathophysiology of PH (Dandel et al.).~The concept of this study is that the elimination of these autoantibodies by Immunoadsorption with protein A may improve haemodynamics and patient wellbeing. Immunoglobulins are substituted after Immunoadsorption to minimize infection risk.
The purpose of this study is to investigate, if Immunoadsorption of autoantibodies with subsequent substitution of immunoglobulins is able to improve haemodynamics in patients with pulmonary hypertension.