Pulmonary Arterial Hypertension: Pathophysiology and Treatment
| Autor: | Norris S H Lan, Benjamin D Massam, Sandeep S Kulkarni, Chim C. Lang |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
medicine.medical_specialty
medicine.drug_mechanism_of_action Prostacyclin Review endothelin receptor antagonists prostacyclin receptor agonists 030204 cardiovascular system & hematology Nitric oxide 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine nitric oxide pulmonary arterial hypertension Internal medicine medicine business.industry prostacyclin-thromboxane prostacyclin analogues medicine.disease mortality Thrombosis Pulmonary hypertension Endothelin 1 medicine.anatomical_structure 030228 respiratory system chemistry Pathophysiology of hypertension endothelin-1 Cardiology Vascular resistance phosphodiesterase-5 inhibitor business Phosphodiesterase 5 inhibitor soluble guanylate cyclase stimulators medicine.drug |
| Zdroj: | Diseases |
| ISSN: | 2079-9721 |
| DOI: | 10.3390/diseases6020038 |
| Popis: | Pulmonary arterial hypertension (PAH), the first category of pulmonary hypertension, is a chronic and progressive disorder characterised by angioproliferative vasculopathy in the pulmonary arterioles, leading to endothelial and smooth muscle proliferation and dysfunction, inflammation and thrombosis. These changes increase pulmonary vascular resistance and subsequent pulmonary arterial pressure, causing right ventricular failure which leads to eventual death if untreated. The management of PAH has advanced rapidly in recent years due to improved understanding of the condition’s pathophysiology, specifically the nitric oxide, prostacyclin-thromboxane and endothelin-1 pathways. Five classes of drugs targeting these pathways are now available: phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, prostacyclin receptor agonists and endothelin receptor antagonists. These developments have led to substantial improvements in mortality rate in recent decades. Recently, long-term studies have demonstrated sustained progression-free survival and have created a new paradigm of initial combination therapy. Despite these targeted therapies, PAH is still associated with significant morbidity and mortality. As such, further research into broadening our understanding of PAH pathophysiology is underway with potential of increasing the repertoire of drugs available. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|