Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model

• Published in: The European respiratory journal Vol. 50; no. 2; p. 1700740
• Main Authors: Hoeper, Marius M., Kramer, Tilmann, Pan, Zixuan, Eichstaedt, Christina A., Spiesshoefer, Jens, Benjamin, Nicola, Olsson, Karen M., Meyer, Katrin, Vizza, Carmine Dario, Vonk-Noordegraaf, Anton, Distler, Oliver, Opitz, Christian, Gibbs, J. Simon R., Delcroix, Marion, Ghofrani, H. Ardeschir, Huscher, Doerte, Pittrow, David, Rosenkranz, Stephan, Grünig, Ekkehard
• Format: Journal Article
• Language: English
• Published: England European Respiratory Society Journals Ltd 01.08.2017
• Subjects: Adult; Aged; Aged, 80 and over; Brain natriuretic peptide; Data processing; Europe - epidemiology; Female; Functional morphology; Heart diseases; Hemodynamics; Humans; Hypertension; Hypertension, Pulmonary - mortality; Kaplan-Meier Estimate; Male; Middle Aged; Mortality; Natriuretic Peptide, Brain - blood; Practice Guidelines as Topic; Prospective Studies; Pulmonary hypertension; Registries; Risk Assessment; Risk factors; Severity of Illness Index; Stratigraphy; Walk Test
• ISSN: 0903-1936; 1399-3003; 1399-3003
• DOI: 10.1183/13993003.00740-2017

The 2015 European pulmonary hypertension (PH) guidelines propose a risk stratification strategy for patients with pulmonary arterial hypertension (PAH). Low-, intermediate- and high-risk strata are defined by estimated 1-year mortality risks of <5%, 5–10% and >10%, respectively. This risk assessment strategy awaits validation. We analysed data from patients with newly diagnosed PAH enrolled into COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension), a European-based PH registry. An abbreviated version of the risk assessment strategy proposed by the European PH guidelines was applied, using the following variables: World Health Organization functional class, 6-min walking distance, brain natriuretic peptide or its N-terminal fragment, right atrial pressure, cardiac index and mixed venous oxygen saturation. Data from 1588 patients were analysed. Mortality rates were significantly different between the three risk strata (p<0.001 for all comparisons). In the entire patient population, the observed mortality rates 1 year after diagnosis were 2.8% in the low-risk cohort (n=196), 9.9% in the intermediate-risk cohort (n=1116) and 21.2% in the high-risk cohort (n=276). In addition, the risk assessment strategy proved valid at follow-up and in major PAH subgroups. An abbreviated version of the risk assessment strategy proposed by the current European PH guidelines provides accurate mortality estimates in patients with PAH.