Abstract
Current European guidelines recommend periodic risk assessment for patients with pulmonary arterial hypertension (PAH). The aim of our study was to determine the association between the number of low-risk criteria achieved within 1 year of diagnosis and long-term prognosis.
Incident patients with idiopathic, heritable and drug-induced PAH between 2006 and 2016 were analysed. The number of low-risk criteria present at diagnosis and at first re-evaluation were assessed: World Health Organization (WHO)/New York Heart Association (NYHA) functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min−1·m−2.
1017 patients were included (mean age 57 years, 59% female, 75% idiopathic PAH). After a median follow-up of 34 months, 238 (23%) patients had died. Each of the four low-risk criteria independently predicted transplant-free survival at first re-evaluation. The number of low-risk criteria present at diagnosis (p<0.001) and at first re-evaluation (p<0.001) discriminated the risk of death or lung transplantation. In addition, in a subgroup of 603 patients with brain natriuretic peptide (BNP) or N-terminal pro-brain natriuretic peptide (NT-proBNP) measurements, the number of three noninvasive criteria (WHO/NYHA functional class, 6MWD and BNP/NT-proBNP) present at first re-evaluation discriminated prognostic groups (p<0.001).
A simplified risk assessment tool that quantifies the number of low-risk criteria present accurately predicted transplant-free survival in PAH.
Abstract
Simplified risk assessment using the number of low-risk criteria predicts prognosis at baseline and follow-up in PAH http://ow.ly/KMsj30cPNbm
Footnotes
This article has supplementary material available from erj.ersjournals.com
Support statement: This work was supported in part by the Assistance Publique-Hôpitaux de Paris (PHRC EFORT: ClinicalTrials.gov Identifier NCT01185730), INSERM, Université Paris-Sud and Agence Nationale de la Recherche (Département Hospitalo-Universitaire Thorax Innovation; LabEx LERMIT, ANR-10-LABX-0033; and RHU BIO-ART LUNG 2020, ANR-15-RHUS-0002). J. Weatherald is the recipient of a joint European Respiratory Society/Canadian Thoracic Society Long-Term Research Fellowship (LTRF 2015 – 4780). Funding information for this article has been deposited with the Crossref Funder Registry.
Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com
- Received May 1, 2017.
- Accepted May 29, 2017.
- Copyright ©ERS 2017