Key result
High-risk ESC/ERS profile predicts ~439% higher mortality vs low-risk in CTEPH patients.
Why the study?
The validity of the 2015 ESC/ERS PAH risk assessment tool for predicting survival in CTEPH patients, considering pulmonary endarterectomy, was uncertain.
Does the 2015 ESC/ERS PAH risk assessment tool predict survival in patients with CTEPH, and how does pulmonary endarterectomy affect this survival?
Cohort (n=250)
Does the 2015 ESC/ERS PAH risk assessment tool predict survival in patients with CTEPH, and how does pulmonary endarterectomy affect this survival?
Hazard Ratio: 5.39 (95% CI 2.13–13.59)
The 2015 ESC/ERS PAH risk assessment tool effectively predicts survival in CTEPH patients, and pulmonary endarterectomy independently improves survival across risk groups.
May guide CTEPH prognostication; extends PAH risk tool but leaves practice change open pending trials.
Objectives To investigate if the pulmonary arterial hypertension (PAH) risk assessment tool presented in the 2015 ESC/ERS guidelines is valid for patients with chronic thromboembolic pulmonary hypertension (CTEPH) when taking pulmonary endarterectomy (PEA) into account. Design. Incident CTEPH patients registered in the Swedish PAH Registry (SPAHR) between 2008 and 2016 were included. Risk stratification performed at baseline and follow-up classified the patients as low-, intermediate-, or high-risk using the proposed ESC/ERS risk algorithm. Results. There were 250 CTEPH patients with median age (interquartile range) 70 (14) years, and 53% were male. Thirty-two percent underwent PEA within 5 (6) months. In a multivariable model adjusting for age, sex, and pharmacological treatment, patients with intermediate-risk or high-risk profiles at baseline displayed an increased mortality risk (Hazard Ratio [95% confidence interval]: 1.64 [0.69–3.90] and 5.39 [2.13–13.59], respectively) compared to those with a low-risk profile, whereas PEA was associated with better survival (0.38 [0.18–0.82]). Similar impact of risk profile and PEA was seen at follow-up. Conclusion. The ESC/ERS risk assessment tool identifies CTEPH patients with reduced survival. Furthermore, PEA improves survival markedly independently of risk group and age. Take home message: The ESC/ERS risk stratification for PAH predicts survival also in CTEPH patients, even when taking PEA into account.
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Sandqvist et al. (2020) conducted a cohort in chronic thromboembolic pulmonary hypertension (CTEPH) (n=250). High-risk profile (ESC/ERS risk assessment tool) vs. Low-risk profile was evaluated on mortality (HR 5.39, 95% CI 2.13-13.59). A high-risk profile by the ESC/ERS risk assessment tool predicted increased mortality in CTEPH patients compared to a low-risk profile (HR 5.39; 95% CI 2.13-13.59).
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