Predicting Survival in Patients With Pulmonary Arterial Hypertension

Abstract

BACKGROUND: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various strategies are available to determine risk status. This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/ Respiratory Society guideline-derived risk assessment strategies. METHODS: A subpopulation from the US-based registry REVEAL that survived $ 1 year postenrollment (baseline for this cohort) was analyzed. For REVEAL 2.0, point values and cutpoints were reassessed, and new variables were evaluated. The Kaplan-Meier method was used to estimate survival at 12 months postbaseline; discrimination was quantified using the c-statistic. Mortality estimates and discrimination were compared between REVEAL 2.0 and Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and French Pulmonary Hypertension Registry (FPHR) risk assessment strategies. For this comparison, a three-category REVEAL 2.0 score was computed in which patients were classified as low-, intermediate-, or high-risk. RESULTS: REVEAL 2.0 demonstrated similar discrimination as the original calculator in this subpopulation (c-statistic ¼ 0.76 vs 0.74), provided excellent separation of risk among the risk categories, and predicted clinical worsening as well as mortality in patients who were followed $ 1 year. The REVEAL 2.0 three-category score had greater discrimination (c-statistic ¼ 0.73) than COMPERA (c-statistic ¼ 0.62) or FPHR (c-statistic ¼ 0.64). Compared with REVEAL 2.0, COMPERA and FPHR both underestimated and overestimated risk. CONCLUSIONS: REVEAL 2.0 demonstrates greater risk discrimination than the COMPERA and FPHR risk assessment strategies in patients enrolled in REVEAL. After external validation, the REVEAL 2.0 calculator can assist clinicians and patients in making informed treatment decisions on the basis of individual risk profiles.

Diagnosis and Management of Pulmonary Hypertension in the Modern Era: Insights from the 6th World Symposium

“…It has been shown to be predictive of survival at baseline [28], at 1-year follow-up [29], and at 5 years [30]. The recently published REVEAL 2.0 calculator has been shown to predict clinical worsening and mortality in patients with PAH who have survived at least 1 year from initial enrollment in the cohort [31]. A European group has also developed a risk-stratification tool using a large PH registry called Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) [32].…”

<p>Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis</p>

“…The 6th World Symposium Proceedings in PH recommend a treatment strategy based on a multiparametric risk stratification approach, 52 with the main objective of achieving a low-risk status that is associated with a reduced mortality (annual mortality of <5%). 20 Several risk stratification strategies have been used, including the REVEAL 2.0, 53 French Pulmonary Hypertension Network (FPHN), 54 (SPAHR), 56 ( Table 4). The two methodologies most commonly used to assess risk are the FPHN and REVEAL 2.0.…”

“…57 The REVEAL 2.0 risk score includes a larger number of variables but provides a greater risk discrimination than the FPHN. 53…”

Real‐world experience using combination therapy with riociguat and risk assessment using REVEAL Lite 2.0 in patients with pulmonary arterial hypertension

“…3 Riociguat is indicated for the treatment of adults with PAH, to improve exercise capacity and World Health Organization (WHO) Functional Class (FC), and to delay clinical worsening. 4,5 In a recent analysis of risk assessment in PAH, REVEAL Lite 1 and 2, which are abridged versions of the REVEAL 2.0 risk score, 3 were shown to be comparable to the full tools. 6 In this report, we present a case series of the use of riociguat in combination or sequentially, and the impact on risk scores as determined by the abridged REVEAL Lite 2.0 approach.…”