Prognostic significance of pericardial effusion in systemic sclerosis-associated pulmonary hypertension: analysis from the PHAROS Registry

Abstract

Objectives: Pulmonary hypertension (PH) is a leading cause of death in patients with SSc. The purpose of this study was to determine the prognostic significance of pericardial effusion in patients with SSc-PH.

Methods: Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) is a prospective multicentre registry which enrolled patients with newly diagnosed SSc-PH from 2005 to 2016. The prognostic impact of pericardial effusion status, including those who ever or never had pericardial effusion, and those who had persistent or intermittent pericardial effusion, was analysed. Kaplan-Meier survival analyses, log-rank test, and multivariable Cox proportional hazards regression were performed.

Results: Of the 335 patients with SSc-PH diagnosed by right heart catheterization and documentation of pericardial effusion presence or absence on echocardiogram, 166 (50%) ever had pericardial effusion. Ever having pericardial effusion was not predictive of survival (log-rank test P = 0.49). Of the 245 SSc-PH patients who had at least two echocardiograms, 44% had a change in pericardial effusion status over an average of 4.3 years of follow up. Having a persistent pericardial effusion was an independent predictor of survival [adjusted hazard ratio (aHR)=2.34, 95% CI 1.20, 4.64, P = 0.002], while intermittent pericardial effusion was not a predictor of survival (aHR = 0.89, 95% CI 0.52, 1.56, P = 0.68), in a multivariable-adjusted analysis.

Conclusion: Persistent pericardial effusion, but not ever having had pericardial effusion or intermittent pericardial effusion, was independently associated with poorer survival. Incorporating information from serial echocardiograms may help clinicians better prognosticate survival in their SSc-PH patients.

Keywords: SSc; pericardial effusion; prospective cohort; pulmonary hypertension.

Figure 1.

Flowchart of patient inclusion for the study. (A) Outlines the flowchart for patients included in the comparative analysis between the ever vs never pericardial effusion groups. (B) Outlines the flowchart for patients included for the comparative analysis among the persistent vs intermittent vs never pericardial effusion groups. PH: pulmonary hypertension

Figure 2.

Kaplan–Meier survival curves for SSc-PH patients enrolled in the PHAROS registry who ever or never had pericardial effusion. Log-rank test P-value = 0.49. PH: pulmonary hypertension

Figure 3.

Sankey diagram of pericardial effusion status changes over time in patients with SSc-PH enrolled in the PHAROS registry

Figure 4.

Kaplan–Meier survival curves for SSc-PH patients enrolled in the PHAROS registry with persistent, intermittent and never pericardial effusion. Log-rank test P-value = 0.002. PH: pulmonary hypertension

Figure 5.

Cumulative incidence function curves of PH-specific and non-PH mortality for SSc-PH patients enrolled in the PHAROS registry with different pericardial effusion status. P-values were calculated by Gray’s test. PH: pulmonary hypertension