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2018 ; 6
(2
): ä Nephropedia Template TP
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English Wikipedia
Pulmonary Arterial Hypertension: Pathophysiology and Treatment
#MMPMID29772649
Lan NSH
; Massam BD
; Kulkarni SS
; Lang CC
Diseases
2018[May]; 6
(2
): ä PMID29772649
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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.