Title:The Innovations in Pulmonary Hypertension Pathophysiology and Treatment: What are our Options!
Volume: 14
Issue: 4
Author(s): Charlene Kalani, Ismael Garcia, Cynthia Ocegueda-Pacheco, Joseph Varon and Salim Surani*
Affiliation:
- Texas A&M University, College Station, Texas,United States
Keywords:
Cor pulmonale, lung transplant, pulmonary hypertension, treatment, pathophysiology, hemodynamic state.
Abstract: Pulmonary hypertension (PH), is a complex and multifactorial entity that affects small
pulmonary vessels. It is defined as an increase in the mean pulmonary arterial pressure (PAPm)
>25mmHg at rest, assessed by right heart catheterization. A recent classification divides PH into five
types: pulmonary arterial hypertension (PAH), PH due to left heart disease, PH due to lung disease
and/or hypoxemia, thromboembolic PH and PH with unclear or multifactorial mechanisms. Several
molecular and biochemical factors are involved in the pathway of this clinical entity, although the
primary origin remains unknown. In recent years, the existence of a genetic predisposition combined
with exposure of environmental factors and triggers has been proposed. In PAH, vasoconstriction,
endothelial dysfunction, thrombosis in situ, remodeling of the pulmonary vessel, inflammation and
cytokines play an important role in the development of this condition. These pathways are the main
targets of the therapeutic interventions in PAH. When feasible, a specific course of action, from drug
therapy to surgical interventions remains the mainstay of survival for these patients. This article
reviews the pathophysiology of this disease as well as available therapeutic options, specifically for
PAH in detail.
