Update on Medical Management of Pulmonary Arterial Hypertension

2022 ◽  
Vol 40 (1) ◽  
pp. 13-27
Author(s):  
Alexander E. Sherman ◽  
Rajan Saggar ◽  
Richard N. Channick
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Pulmonary Arterial

Lung transplantation in pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2520-2524
Author(s):  
Laurent Savale
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Medical Management ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Right Heart Failure ◽  
Optimal Timing ◽  
Optimal Medical Management ◽  
Pulmonary Arterial

Despite recent advances in medical management, lung and heart–lung transplantation remains an established treatment option in patients with end-stage pulmonary arterial hypertension (PAH) despite optimal medical therapy. The optimal timing of referral and listing patients for transplantation must take into consideration the co-morbidities, the aetiology of PAH, and the regular risk assessment based on a multidimensional approach. With the exception of PAH due to complex congenital heart disease, double-lung transplantation is the preferred option over combined heart–lung transplantation as cardiac function recovers after transplantation in most cases. The use of extracorporeal life support as a bridge to urgent transplantation may be considered in expert centres in selected patients with refractory right heart failure despite optimal medical management in an intensive care unit. With a survival rate at 5 years varying from 50% to 75%, transplantation in PAH can be considered as a life-saving procedure for patients with severe symptoms.

Medical Management of Pulmonary Arterial Hypertension

2018 ◽  
Author(s):  
Inderjit Singh ◽  
Aaron B Waxman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Endothelin Receptor ◽  
Prostacyclin Receptor ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

The medical management of pulmonary arterial hypertension (PAH) has advanced considerably over the years. Once the diagnosis of PAH is made, the medical management includes both conventional and PAH-pathway specific therapies. Five different classes of drugs are now available targeting the endothelin, prostacyclin, and nitric oxide pathways (i.e. endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists). These targeted therapies are approved for treatment of patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH) only. Long-term and event-driven studies of novel drugs have led to further improvement in the medical management of PAH and CTEPH. In this review, we will focus on the medical management of patients with PAH and CTEPH. This review contains 4 figures, 6 Tables and 30 references Keywords: Pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Medical management, Pulmonary vasodilators, Phosphodiesterase inhibitor, Soluble guanylate cyclase stimulator, Endothelin receptor antagonist, Prostacyclin receptor agonist

Medical Management of Pulmonary Arterial Hypertension

2018 ◽  
Author(s):  
Inderjit Singh ◽  
Aaron B Waxman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Endothelin Receptor ◽  
Prostacyclin Receptor ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

The medical management of pulmonary arterial hypertension (PAH) has advanced considerably over the years. Once the diagnosis of PAH is made, the medical management includes both conventional and PAH-pathway specific therapies. Five different classes of drugs are now available targeting the endothelin, prostacyclin, and nitric oxide pathways (i.e. endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists). These targeted therapies are approved for treatment of patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH) only. Long-term and event-driven studies of novel drugs have led to further improvement in the medical management of PAH and CTEPH. In this review, we will focus on the medical management of patients with PAH and CTEPH. This review contains 4 figures, 6 Tables and 30 references Keywords: Pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Medical management, Pulmonary vasodilators, Phosphodiesterase inhibitor, Soluble guanylate cyclase stimulator, Endothelin receptor antagonist, Prostacyclin receptor agonist

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