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]
Adapted from Hoeper et al. 2017[5]
PAH, pulmonary arterial hypertension
Clinical guidelines set out the diagnostic pathway so patients can be referred and treated as quickly as possible.
While the exact cause of PAH is uncertain, a clearer picture of the underlying pathological mechanisms is emerging.
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