Pulmonary arterial hypertension (PAH) is a rare and severe disease that continues to progress over time. Although there is no cure, recent advances in treatment strategies with increased therapeutic options have offered an improvement in prognosis and survival.,
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. Patients across all risk categories have poor survival; therefore, treatment strategies should target the improvement of long-term outcomes, with the ultimate goal of achieving a low-risk status.
Adapted from Hoeper et al. 2017
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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