Prognostic Implications of Pulmonary Arterial Hypertension-Related Morbidity

Study Questions:

What is the impact of pulmonary arterial hypertension (PAH)-related morbidity events on the risk of subsequent mortality using the landmark method and data from the SERAPHIN and GRIPHON studies?


The investigators included patients from SERAPHIN and GRIPHON trials in the analyses. For each study, the risk of all-cause death up to end of study was assessed from the landmark time point (months 3, 6, and 12) according to whether a patient had experienced a primary endpoint morbidity event prior to the landmark. Each analysis was conducted using data from all patients who were available for survival follow-up at the landmark.


In SERAPHIN, based on the 3-month landmark time point, patients who experienced a morbidity event prior to month 3 had an increased risk of death compared with patients who did not (hazard ratio [HR], 3.39; 95% confidence interval [CI], 1.94-5.92). In GRIPHON, based on the 3-month landmark time point, there was also an increased risk with an HR of 4.48 (95% CI, 2.98-6.73). Analyses based on 6- and 12-month landmarks also showed increased risk in patients who experienced morbidity events, albeit with a reduced hazard ratio.


The authors concluded that the results demonstrate the prognostic relevance of PAH-related morbidity, highlighting the importance of preventing disease progression in patients with PAH.


This study suggests that PAH-related morbidity, as captured in the primary endpoint events of the SERAPHIN and GRIPHON studies, is prognostic for mortality. These findings are supported by consistent results in two independent studies and at three different time points within each of the studies. Furthermore, the analyses of the GRIPHON data also highlight the relevance of hospitalization for worsening of PAH as a risk factor for subsequent mortality. Overall, these results point out the risk associated with disease progression in PAH and underscore the need for treatment regimens and management strategies that prevent deterioration.

Clinical Topics: Heart Failure and Cardiomyopathies, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Pulmonary Hypertension, Hypertension

Keywords: Disease Progression, Hypertension, Pulmonary, Hypertension, Morbidity, Mortality, Primary Prevention, Prognosis, Pulmonary Artery, Risk Factors

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