Physicians' Academy for Cardiovascular Education

Low-risk profile in pulmonary arterial hypertension patients predicts better survival

A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension

Kylhammar D, Kjellström B, Hjalmarsson C, et al. - Eur Heart J 2017, In press


Despite advances in drug therapy that target the endothelin, nitric oxide and prostacyclin pathways, pulmonary arterial hypertension (PAH) has still a poor prognosis [1-3]. Measures at baseline and during follow-up that reflect right ventricular performance, predict outcome [3-5]. Treatment protocols advocate a goal-oriented approach, meaning to achieve a low-risk profile preferably based on functional class (FC), exercise capacity and right ventricular function, which is included in a risk assessment instrument presented in the ESC/ERS guidelines as of 2009 [6,7].

It is possible that variables that are included in this risk assessment instrument predict prognosis during follow-up, however this is incompletely validated (level of evidence C) [4,5]. The goal of this study was to test the discriminatory ability of the risk assessment instrument, using the Swedish PAH register (SPAHR, started in 2008 [8]), and to explore the potential prognostic advantage of a low-risk profile at follow-up which was performed within one year from baseline. For this, 7 variables per patient were assessed at baseline and 5 during follow-up. The study included 261 patients with idiopathic PAH (IPAH), 7 with familiar PAH (FPAH), 162 with connective tissue disease-associated PAH (APAH-CTD), 67 with coronary heart disease-associated PAH (APAH-CHD), 16 with drugs- and toxins-induced PAH, 3 with HIV-associated PAH and 14 with portal hypertension-associated PAH. Median time to first follow-up was 4 months and total follow-up 27 months.

Main results


These results support the use of comprehensive risk assessments and goal-oriented treatment approach towards a low-risk profile, as proposed by the ESC/ERS guidelines 2015. Moreover, the fact that only 29% of patients were in the low-risk group at follow-up, urges for better treatments and treatment strategies in general. Furthermore, the risk assessment successfully discriminates patients with better or worse outcome both at baseline and at follow-up, in which determination at follow-up better predicts long-term outcome.


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Find this article online at Eur Heart J