Fig. 5.1 Proposed intra- and inter-cellular signaling mechanisms involved in fibroblast-macrophage crosstalk in chronic non-resolving perivascular inflammation underlying irreversible pulmonary vascular remodeling. In this process, the adventitial fibroblast undergoes epigenetic…
Fig. 6.1 Molecular pathogenesis of hereditary PAH. BMP binds to BMPR2. Upon ligand binding, the type II receptor phosphorylates a type I receptor, including ALK1, ALK2, ALK3 or ALK6. This…
Fig. 16.1 Pulmonary artery smooth muscle cell therapeutic targets and corresponding licensed (green boxes) and investigational (red boxes) treatment approaches for pulmonary arterial hypertension. Arrows represent receptor stimulation, whereas terminated…
Hypoxic signaling HIF-1α/HIF-2α TGF-β ERK1/2 Increased Ca++ Mitochondrial dysfunction Mitochondrial hyperpolarization Suppressed mitophagy Increased mROS generation HIF-1α and aerobic glycolysis Quasi malignancy Clonal endothelial cell proliferation Microsatellite instability Somatic chromosomal…
Fig. 21.1 The pulmonary hypertension clinical patient care continuum. The Pulmonary Hypertension Clinical (PHC) care coordinator plays a pivotal role in coordinating each element of the treatment plan individualized to…
Fig. 18.1 Example of a potential induction therapy protocol. If a similar protocol were to be transformed into a clinical trial, an additional arm could include dual oral therapy. A…
Fig. 4.1 Group specific prevalence of pulmonary hypertension Temporal trends in mortality and hospitalization where PH was listed as any contributing cause of death or any-listed hospital diagnosis in the…
Biomarker Study endpoints Endothelins Endothelin-1 Hemodynamic measurements, survival Big ET-1 Hemodynamic measurements, 6MWD ET-1/ET-3 Hemodynamic measurements, NYHA FC, survival CT-pro-ET-1 Clinical severity, hospitalizations, survival vWF Hemodynamic measurements, survival Angiopoietin Hemodynamic…
Fig. 10.1 Pulmonary arterial hypertension time-course and type of vascular remodeling pattern secondary to exposure to the monocrotraline pyrrole. A single subcutaneous injection of monocrotaline at a dose of 60…
Fig. 8.1 Metabolic pathways reprogrammed in PAH. Several pathways are affected in humans with and experimental models of PAH. Increased glycolysis, lactate fermentation, and glutaminolysis are observed (purple, pink, and…
