Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

TLDR

Current European guidelines recommend periodic risk assessment for patients with pulmonary arterial hypertension (PAH). This study aimed to determine whether the number of low‑risk criteria achieved within one year of diagnosis predicts long‑term prognosis. We analysed incident idiopathic, heritable and drug‑induced PAH patients from 2006‑2016 and evaluated the number of low‑risk criteria—WHO/NYHA functional class I/II, 6‑min walk distance >440 m, right atrial pressure <8 mmHg, and cardiac index ≥2.5 L·min⁻¹·m⁻²—at diagnosis and first re‑evaluation. Among 1,017 patients (median 34 months follow‑up), each low‑risk criterion independently predicted transplant‑free survival, and the total number of criteria at diagnosis and first re‑evaluation (including a simplified three‑criterion tool) accurately discriminated risk of death or lung transplantation.

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) &gt;440 m, right atrial pressure &lt;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&lt;0.001) and at first re-evaluation (p&lt;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&lt;0.001). A simplified risk assessment tool that quantifies the number of low-risk criteria present accurately predicted transplant-free survival in PAH.