Fig. 15.1 Simplified representation of “unfolded circulation” with representation of right atrium (RA), right ventricle (RV), lungs, left atrium (LA), left ventricle (LV) and systemic organs. Concepts related to coupling…
Registry Time period Year of publication Method of data collection Location (number of centers) Number of patients NIH* [5] 1981–1985 1987 prospective United States (32) 187 French National* [10] 2002–2003…
Knowledge gap Strategy Example of present or future state Unifying theory of PH across WHO groups Cross-sectional deep phenotyping; consensus phenotypic definitions NHLBI PVD phenomics; consensus documents [1] Predicting PAH…
Fig. 22.1 Capillarization is increased in quadriceps muscle in one PH patient (a) before and (b) after exercise training (Adapted from de Man et al.[8]; Reproduced with permission of the…
Fig. 17.1 Two approaches to initiation of therapy in PAH: sequential combination therapy or up-front combination therapy A. Sequential Combination Therapy. Treatment is initiated with monotherapy and the outcome assessed….
Vessel type Artery Mean α1-AR density (fmol/mg protein) Arteries Central Aorta 9.8 ± 1.8 Small somatic Mammary 6.0 ± 1.3 Splanchnic Mesenteric 13 ± 6.1 Splenic 28 ± 7.7 Hepatic 28 ± 5.9 Renal Renal 23 ± 5.9 Pulmonary Pulmonary artery 24 ± 5.2…
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…
