Background and Importance The 2015 and 2022 ESC/ERS Guidelines for pulmonary hypertension treatment provide algorithms for decision-making based on patients’ 1-year mortality risk, with strong recommendations to intensify treatment in patients with intermediate-high risk.
Aim and Objectives To assess whether treatment decisions in pulmonary arterial hypertension [PAH] patients are currently being made according to the treatment algorithms provided by the ESC/ERS Guidelines.
Material and Methods A retrospective, descriptive, cross-sectional (March 2022) study was carried out in 2 tertiary hospitals, including alive adult PAH patients who initiated a PAH-specific therapy after 2016 and whose medical charts provided enough data to estimate the risk of 1-year mortality with the simplified four-strata risk-assessment tool.
Medical charts were consulted in order to collect several variables: demographic data, PAH subclassification according to aetiology, PH-specific drug initiated, World Health Organization functional class [WHO-FC], 6-minute walking distance [6MWD], and N-terminal pro-brain natriuretic peptide [NT-proBNP].
1-year mortality risk and the appropriateness of PH-specific therapies prescribed were assessed according to PAH treatment algorithms provided by the 2015 and 2022 ESC/ERS Guidelines.
Results 37 patients complied with inclusion criteria, 54.1% women aged 50 (28–84).
Patients’ HAP subsets: 14, 6, 2, 2, and 1 were associated with adult congenital heart disease, portal hypertension, connective tissue disease, drugs and toxins, and human immunodeficiency virus infection, respectively. 6 patients were classified as idiopathic HAP.
52 changes in pulmonary-specific therapy were carried out in the studied period. At treatment initiation patients:
WHO-FC: I, II, III, and IV in 2, 21, 26, and 3 cases, respectively.
6MWD: 425 (146–760) metres
NT-proBNP: 369 (12–7200) ng/L
Risk: 17 low, 20 intermediate-low, 14 intermediate-high, and 1 high.
36/52 treatment initiations were adequate according to clinical guideline algorithms; most discrepancies were due to:
- Initiation of selexipag (n=9) or riociguat (n=3) in patients with risk other than intermediate-low.
Conclusion and Relevance In this cohort of PAH patients whose 1-year mortality risk could be estimated, treatment decisions were generally made according to treatment guidelines.
Patients‘ preferences could explain most discrepancies, as they may prioritise avoiding treatments that require parenteral administration, such as epoprostenol and treprostinil and rather try oral alternatives.
Conflict of Interest No conflict of interest
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