Novel treatment targets for pulmonary arterial hypertension and right ventricular failure

Pulmonary hypertension (PH) is a disease characterized by an increased blood pressure in the pulmonary vascular system, ultimately leading to right ventricular failure (RVF) and premature death. Pulmonary arterial hypertension (PAH), PH group, is defined by an increase in mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, pulmonary wedge pressure ≤ 15 mmHg, and pulmonary vascular resistance > 3 wood units measured by right heart catheterization. This thesis aimed to investigate novel promising treatment strategies for PAH and PAH induced RVF, based on multiple pathological mechanisms underlying the disease. I investigated drugs impacting on the bone morphogenetic protein type 2 receptor (BMPR2) signaling pathway (Chapter 2-4), on growth factor signaling (Chapter 5), on histone acetylation (Chapter 6), as well as several therapeutic strategies specifically targeting the RV (Chapter 7-9).