Longitudinal changes in risk status in pulmonary arterial hypertension

Abstract

Aims Low-risk status in pulmonary arterial hypertension (PAH) predicts better survival. The present study aimed to describe changes in risk status and treatment approaches over multiple clinical assessments in PAH, taking age and comorbidity burden into consideration. Methods and results The study included incident patients from the Swedish PAH registry, diagnosed with PAH in 2008-2019. Group A (n = 340) were ≤75 years old with <3 comorbidities. Group B (n = 163) were >75 years old with ≥3 comorbidities. Assessments occurred at baseline, first-year (Y1) and third-year (Y3) follow-ups. The study used an explorative and descriptive approach. Group A: median age was 65 years, 70% were female, and 46% had no comorbidities at baseline. Baseline risk assessment yielded low (23%), intermediate (66%), and high risk (11%). Among patients at low, intermediate, or high risk at baseline, 51%, 18%, and 13%, respectively, were at low risk at Y3. At baseline, monotherapy was the most common therapy among low (68%) and intermediate groups (60%), while dual therapy was the most common among high risk (69%). In patients assessed as low, intermediate, or high risk at Y1, 66%, 12%, and 0% were at low risk at Y3, respectively. Of patients at intermediate or high risk at Y1, 35% received monotherapy and 13% received triple therapy. In low-risk patients at Y1, monotherapy (40%) and dual therapy (43%) were evenly distributed. Group B: median age was 77 years, 50% were female, and 44% had ≥3 comorbidities at baseline. At baseline, 8% were at low, 80% at intermediate, and 12% at high risk. Monotherapy was the most common therapy (62%) in Group B at baseline. Few patients maintained or reached low risk at follow-ups. Conclusions Most patients with PAH did not meet low-risk criteria during the 3 year follow-up. The first year from diagnosis seems important in defining the longitudinal risk status.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…The main limitation of the 2015 ESC/ERS three-strata, risk-assessment tool is that 60-70% of the patients are classified as intermediate risk [274,[293][294][295][297][298][299][300][301][302][303]. An initial attempt to substratify the intermediate-risk group has been proposed, using a modified mean score in the SPAHR equation (with low-intermediate, 1.5-1.99 and high-intermediate, 2.0-2.49 as cut-offs), where the high-intermediate group was associated with worse survival [302].…”

Characteristics, goal‐oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry

“…[7][8][9][10][11][12][13] However, investigations regarding longitudinal changes in goal-oriented treatments are still scarce in real-world registries, which might cloud better optimization of treatment strategies. 14 The applicability of risk assessment tools also remains to be explored and verified in independent cohorts.…”

“…The benefits of maintaining or achieving a low‐risk profile have been stressed in several studies utilizing different risk stratifications, with a goal‐oriented treatment strategy recommended for PAH management 7–13 . However, investigations regarding longitudinal changes in goal‐oriented treatments are still scarce in real‐world registries, which might cloud better optimization of treatment strategies 14 . The applicability of risk assessment tools also remains to be explored and verified in independent cohorts.…”

Expert consensus on the stratification, management, and follow-up of patients with Group 1 pulmonary hypertension at intermediate risk

“…Stratification is essential within the first year after diagnosis, since the risk status during that year appears to be critical in predicting the risk status the patient will achieve longterm. 11,12 The clinical management guidelines and expert consensuses suggest determining clinical deterioration, progression or concomitant diseases, RV function (along with its stability or sufficiency), the patient's current status and his/her long-term prognosis. 2 This is more feasible using a multidimensional approach, which would ideally measure exercise capacity and provide RV data.…”