Therapeutic Advances in the Management of Pulmonary Arterial Hypertension

Document Type

Article

Publication Title

Chronic Lung Diseases: Pathophysiology and Therapeutics

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease with multiple etiologies. If remains untreated, it leads to high rate of morbidity and mortality, and a median survival of only 5-7 years. Clinically, PAH is defined as mean pulmonary arterial pressure >20 mmHg at rest with normal left atrial pressure. PAH is characterized by remodeling of the pulmonary vasculature due to pulmonary vascular endothelial and smooth cell proliferation. These events eventually reduce the pulmonary arterial compliance and thus causing increased pulmonary vascular resistance. If remains unmanaged, it will ultimately end up in right ventricular failure. However, the Food and Drug Administration (FDA) has approved drugs that target the endothelial pathway, the nitric oxide pathway, and the prostacyclin pathway. These therapeutic strategies, mainly inducing pulmonary vasodilation, however, have poor effect on the signaling pathways activated during the pathogenesis of PAH. This might be one of the reasons why they are unable to reverse the pathology of the disease, and eventually patients stop responding to them. If medications fail, the only viable alternative will be lung transplantation to save a patient’s life. Evidences show the contribution of heredity, inflammation, drugs, toxins, HIV infection, and hepatic diseases in the progression of PAH. Therefore, there is a need for potential therapeutics, which, via affecting the pathological signaling pathways, halt the disease process and eventually reverse the pulmonary remodeling in the patients. In this chapter, we discuss the emerging novel therapeutics and their mechanism of actions, which have advanced beyond preclinical research and are currently being investigated in the clinical cases of PAH.

First Page

1

Last Page

23

DOI

10.1007/978-981-15-3734-9_1

Publication Date

1-1-2020

This document is currently not available here.

Share

COinS