Assessing risk in pulmonary arterial hypertension: what we know, what we don't

Despite improvement in short-term survival, pulmonary arterial hypertension (PAH) remains an incurable disease with an unacceptable median survival of 7 years [1]. In the USA, the survival rates for PAH patients awaiting transplant continue to mirror the 2.5-year survival rate in the pretreatment era of the disease [2]. Despite treatment, PAH patients continue to experience disease progression and increased rates of hospitalisations due to right heart failure. Importantly, right heart failure hospitalisations in PAH occur at rates that are comparable to patients with left heart disease, particularly in those aged greater than 65 [3] More so, morbid events in PAH, notably hospitalisations, herald disease progression and early mortality [4, 5]. Hence, along with advancing PAH treatment options, appropriate and accurate risk prediction is essential to halt disease progression and make individualised treatment decisions. Risk stratification in PAH is best served by joint use of the ERS/ESC risk table and REVEAL calculator risk tools <http://ow.ly/ySrB30dKq9r>