Predicting mortality during long-term follow-up in pulmonary arterial hypertension

Abstract

The European Society of Cardiology (ESC) and European Respiratory Society (ERS) guideline recommendation of comprehensive risk assessments, which classify patients with pulmonary arterial hypertension (PAH) as having low, intermediate or high mortality risk, has not been evaluated during long-term follow-up in a “real-life” clinical setting. We therefore aimed to investigate the utility of risk assessment in a clinical setting for up to 5 years post diagnosis.Three hundred and eighty-six patients with PAH from the Swedish PAH Registry were included. Risk group (low/intermediate/high) and proportion of low risk variables were investigated at 3-, 4- and 5-year follow-ups after time of diagnosis. In an exploratory analysis, survival rates of patients with low- or high intermediate risk scores were compared.A low risk profile was in multivariate Cox proportional hazards regressions found to be a strong, independent predictor of longer transplant-free survival (p<0.001) at the 3-, 4- and 5-year follow-ups. Also, for the 3-, 4- and 5-year follow-ups, survival rates significantly differed (p<0.001) between the three risk groups. Patients with a greater proportion of low risk variables had better (p<0.001) survival rates. Patients with a high intermediate risk score had worse survival rates (p<0.001) than those with a low intermediate risk score. Results were similar when excluding patients with ≥3 risk factors for heart failure with preserved ejection fraction, atrial fibrillation and/or age >75 years at diagnosis.Our findings suggest that the ESC/ERS guideline strategy for comprehensive risk assessments in PAH is valid also during long-term follow-up in a “real-life” clinical setting.

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]. There have also been attempts to further improve risk stratification by exploring the additional value of new biomarkers [304], or by measuring RV structure and function by echocardiography and cMRI [231,305,306].…”

“…c Observe that 6MWD is dependent upon age, height, and burden of comorbidities. d To harmonize with the four-strata model shown in Table18, the BNP and NT-proBNP cut-off levels have been updated from the 2015 version based on data from the REVEAL registry, acknowledging that the European validation studies have used the original cut-off levels[274,292,293,295,296,302]. e cMRI parameters adapted from Section 6.2.2.2. https://doi.org/10.1183/13993003.00879-2022…”

“…Class a Level bIt is recommended to evaluate disease severity in patients with PAH with a panel of data derived from clinical assessment, exercise tests, biochemical markers, echocardiography, and haemodynamic evaluations[212,213,216,249,292,293,295,296,302,307] …”

COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension

“…Several investigators have shown that the use of additional variables derived from echocardiography, right heart catheterization or blood gas analysis improved risk prediction [11][12][13][14]. As an alternative model, the SPAHR group recently proposed a modification of their original approach defining a calculated score of 1.5-1.99 as intermediate-low risk and a score of 2.0-2.4 as intermediate-high risk [15]. This approach showed promising results with further discrimination within the intermediate-risk group, albeit based on a relatively small sample size.…”

Anxiety and Depression in Patients With Pulmonary Arterial Hypertension in Northwest China: A Cross-Sectional Study

“…These changes can significantly affect psychological and physical conditions of patients in different ways. For example, PAH patients may have feelings of uncertainty about their future, significant economic burden (7), a high risk of maternal mortality (30-55%) (7,8), and heavy side effects of medical therapies (9). All of these could have a negative effect on a patient's mental status, resulting in the occurrence of depression and anxiety symptoms.…”