1. Introduction


2. Classification of pulmonary hypertension


3. Epidemiology of pulmonary hypertension
3.1 Pulmonary arterial hypertension (PAH)
3.2 PH due to left heart disease
3.3 PH due to lung disease
3.4 Chronic thromboembolic pulmonary hypertension (CTEPH)
3.5 PH with unclear multifactorial mechanisms
3.6 PH in the pediatric population


4. Pathophysiology of pulmonary hypertension
4.1 Pathophysiology of pulmonary arterial hypertension (PAH)
4.2 Pathophysiology of PH due to left heart disease (PH-LHD)
4.3 Pathophysiology of PH due to lung diseases and/or hypoxia
4.4 Pathophysiology of chronic thromboembolic pulmonary hypertension (CTEPH)
4.5 Pathophysiology of PH with unclear multifactorial mechanisms
4.6 Inflammation and immunity in the pathogenesis of PH
4.7 The right ventricle (RV) in PH


5. Mechanisms of vascular remodeling in PH
5.1 Altered ion channel activity
5.2 Endothelial smooth muscle cell interactions
5.3 Aberrant growth factor signaling
5.4 Hypoxia and hypoxia-inducible factors (HIFs)
5.5 Extracellular vesicles (EVs)
5.6 Role of microRNAs (miRNAs)
5.7 The genetic variants and epigenetic changes associated with PH
5.8 Role of the vascular adventitia


6. Mechanisms of excessive pulmonary vasoconstriction in PH
6.1 Diminished EDNO-cGMP signaling
6.2 Diminished prostacyclin-cAMP signaling
6.3 Exaggerated ET-1 and Rho kinase signaling
6.4 Role of reactive oxygen species (ROS)
6.5 Role of metabolism


Concluding remarks
References
Author biographies.