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Jason Weatherald, Olivier Sitbon, Marc Humbert, Validation of a risk assessment instrument for pulmonary arterial hypertension, European Heart Journal, Volume 39, Issue 47, 14 December 2018, Pages 4182–4185, https://doi.org/10.1093/eurheartj/ehx301
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This editorial refers to ‘A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension’†, by D. Kylhammar et al., on page 4175.
In this issue of the journal, Kylhammar et al.1 aimed to validate the pulmonary arterial hypertension (PAH) risk stratification instrument presented in the 2015 ESC/ERS guidelines.2 , 3 For 530 patients from the Swedish PAH registry, they calculated a risk score by assigning a grade from 1 to 3 for each available variable according to the risk level of that variable (low risk = 1, intermediate risk = 2, high risk = 3) and obtaining the mean grade for each patient, rounded to the nearest integer, thereby defining their risk group (low, intermediate, or high) at baseline. They then recalculated the mean grade at the time of follow-up for 383 patients who had a follow-up assessment within 1 year of diagnosis. At baseline, the vast majority of patients were intermediate risk (67%), with 23% classified as low risk and 10% as high risk. Surprisingly, there was little change in the proportions of patients who were low risk (29%), intermediate risk (60%) or high risk (11%) at follow-up, after PAH treatment was initiated. They found that the application of their risk score approach predicted the 1-, 3-, and 5-year risk of death at baseline and follow-up. This held true in important subgroups with idiopathic, familial, and connective tissue disease-associated PAH, and when older idiopathic PAH patients (>65 years old) were excluded. The 1-year mortality rates in the low-, intermediate-, and high-risk groups at baseline were 1, 17, and 26%, respectively, with a mortality risk in the intermediate group somewhat higher than the expected 5–10% in the guidelines.2 , 3 Repeating the risk assessment at follow-up, the 1-year mortality rates for low-, intermediate-, and high-risk patients were 1, 9, and 30%, which correspond well to the estimates proposed in the guidelines. They also confirmed previous findings by demonstrating the importance of improvements and maintenance of low-risk features during follow-up.4 , 5 Patients who worsened from low risk at baseline to intermediate or high risk at follow-up had much worse survival than those who were intermediate or high risk at baseline and improved to a low-risk profile. Perhaps most importantly, survival consistently improved as an increasing proportion of available variables at follow-up were in the ‘low-risk’ profile.
