What Can Be Done To Clinically Diagnose Pulmonary Arterial Hypertension Early?
Pulmonary arterial hypertension (PAH) is slow to diagnose because the symptoms of the
disease mimic those of more common
diseases and may be mild to nonexistent in the early stages of the
Early Diagnosis is Key in Pulmonary Arterial Hypertension Management
PAH is a progressive, potentially life-threatening disease that
affects the lungs and subsequently the
*Data are from the Patient Registry for the Characterization of Primary Pulmonary Hypertension, initiated by the National Institute of Health in 1981. This multicenter, prospective registry included 32 medical centers in the United States with 187 patients with primary pulmonary hypertension enrolled from July 1981 to September 1985. Limitations include lack of standardized follow-up assessments; prospective studies are needed to validate findings.
†Data are from the Registry to Evaluate Early and Long-term PAH disease management (REVEAL Registry), a large, multicenter, prospective cohort registry that included 54 centers in the United States. 2,967 patients were enrolled between March 2006 and September 2007, all with newly or previously diagnosed World Health Organization group I PAH and pre-specified hemodynamic criteria by right-heart catheterization test. Limitations include lack of standardized follow-up assessments; prospective studies are needed to validate findings.
Implementing an Effective Risk Assessment Strategy in Pulmonary Arterial Hypertension
What Are the Pharmacologic Approaches for PAH Management?
There are multiple pathophysiologic pathways that have been implicated in the pathogenesis of PAH with current
therapies focusing on the imbalance of vasoconstriction and vasodilation (prostacyclin
Nitric oxide pathway
PDE-5 inhibitors increase cGMP, while soluble guanylate cyclase stimulators enhance cGMP production.
Both induce vasodilation
Prostacyclin pathway
Prostacyclin-class therapies result in vasodilation and inhibition of platelet aggregation and smooth muscle cell proliferation
Endothelin pathway
Endothelin receptor antagonists prevent vasoconstrictive and endothelium proliferative effects
cGMP=cyclic guanosine monophosphate; PDE-5=phosphodiesterase type 5.
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