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Diagnosis of PAH

Treatment of Pulmonary Arterial Hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a rare and severe disease that continues to progress over time.[1] Although there is no cure, recent advances in treatment strategies with increased therapeutic options have offered an improvement in prognosis and survival.[2],[3]

The prognosis of PAH depends on a number of modifiable clinical, functional, exercise, biochemical, echocardiographic and haemodynamic variables. Comprehensive assessment based on these parameters can determine prognosis by classifying patients as low-risk, intermediate-risk or high-risk for clinical worsening or death.[4] Patients across all risk categories have poor survival;[5] therefore, treatment strategies should target the improvement of long-term outcomes, with the ultimate goal of achieving a low-risk status.[4]

PAH-Explained-Treatment - Prognosis

Adapted from Hoeper et al. 2017[5]
PAH, pulmonary arterial hypertension

Diagnosis of PAH

Clinical guidelines set out the diagnostic pathway so patients can be referred and treated as quickly as possible.

Pathophysiology of PAH

While the exact cause of PAH is uncertain, a clearer picture of the underlying pathological mechanisms is emerging.

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References

D’Alonzo GE et al. Ann Intern Med 1991; 115:343–349.
Benza RL et al. Chest 2012; 142:448–456.
Montani D et al. Orphanet J Rare Dis 2013; 8:97.
Galiè N et al. Eur Heart J 2016; 37:67–119.
Hoeper MM et al. Eur Respir J 2017; 50:1700740.
Boucly A et al. Eur Respir J 2017; 50:1700889.
Humbert M et al. Circulation 2014; 130:2189–2208.
Rosenkranz S, Dumitrescu D. Rev Esp Cardiol (Engl Ed) 2017; 70:901–904.
Lajoie A et al. Pulm Circ 2017; 7:312–325.
CP-192318 - November 2020