Risk assessments are based on registries
Multiple registries from across the globe track thousands of patients with pulmonary arterial hypertension (PAH). Because of the depth and breadth of information collected in worldwide PAH registries, registry data are the foundation of today’s treatment guidelines.1,2 The data are consistent: Patients who achieve low-risk status within their first year after diagnosis were shown to have a better likelihood of survival. Formal risk calculation methods based on these registry data can help you predict your patients’ chance of survival at 5 years.3-8
What are the benefits of using registry data?
Patient registries contain a wealth of real-world information and allow for discoveries of patterns outside the strict confines of RCTs. Robust registries focus on a specified population of patients, for example, patients with PAH, while collecting prespecified data from multiple institutions. Registries collect data on thousands of patients over time—far longer than RCTs—thus following large numbers of patients throughout their PAH journey.9
Generalized limitations of registry data
PAH risk assessment methods are based on large patient registry data sets. To use these registries thoughtfully, limitations of registry data should be acknowledged.
A few limitations to consider when reviewing registry data analysis include7,8,10:
- Limited generalization, depending on the patient population and the size of the registry
- Dependent on data collected, not the data that may be needed for a study
- Historical data, some of which may be bypassed for updated knowledge and therapies
- Potential need for prospective, external validation
US REVEAL Registry
The US-based REVEAL Registry supports the prognostic utility of risk stratification.6-8 REVEAL 2.0 and REVEAL Lite 2 risk calculators can be used to calculate your patients’ risk status.6-8 REVEAL-ECHO calculations include updates that emphasize the importance of Echo measurements for an accurate prognosis and critical risk assessment results.10
Overview
US multicenter, retrospective refinements of study (2006-2013); N=2529
US multicenter, retrospective echocardiographic data (2006-2013); N=2400
Variables
13 variables*:
- WHO Group 1 subgroup
- Age/sex
- Renal insufficiency
- NYHA/WHO FC
- SBP
- HR
- 6MWD
- BNP/NT-proBNP
- Pericardial effusion
- DLCO
- mRAP
- PVR
- All-cause hospitalizations ≤6 months
6 noninvasive variables†:
- Renal insufficiency
- NYHA/WHO FC
- SBP
- HR
- 6MWD
- BP/NT-proBNP
4 echocardiographic parameters:
- RV chamber enlargement
- RV reduced systolic function
- Tricuspid regurgitation severity
- Pericardial effusion
- PAH etiology subgroup
Risk calculation
Weighted scores assigned to each value based on the variable’s contribution to risk
Risk stratification:
- Low-risk score (≤6)
- Intermediate-risk score (7-8)
- High-risk score (≥9)
Weighted scores assigned to each value based on the variable’s contribution to risk
Risk stratification:
- Low-risk score (≤5)
- Intermediate-risk score (6-7)
- High-risk score (≥8)
Weighted scores assigned to each value based on the variable’s contribution to risk
Risk stratification:
- Low-risk score (0-1)
- Intermediate-risk score (2-3)
- High-risk score (4-10)
Suggested use2,8,10
Baseline risk assessment
Annual risk assessment
Follow-up risk assessment
Follow-up risk assessment
Potential complementary use with other risk tools‡
European registries
European registries involving more than 3000 patients also support the prognostic utility of risk stratification.3-5 Online calculators developed from these registries, ESC/ERS Treatment Guidelines, COMPERA 2.0: 4-Risk Strata, and French Noninvasive Criteria, can be used to calculate your patient’s risk status.
Overview
European prospective observational study (2009-2016); N=1588
European prospective observational study (2009-2020); N=1655
Variables
6 variables:
- WHO FC
- 6MWD
- RAP
- CI
- NT-proBNP or BNP
- SvO2
3 variables:
- WHO FC
- 6MWD
- NT-proBNP or BNP
Risk calculation
Assigned risk-category grades to each variable based on ESC/ERS Treatment Guidelines (1=low, 2=intermediate, 3=high)
Risk category obtained by dividing sum of all grades by number of available variables and rounding to nearest integer
Assigned risk-category grades to each variable with cutoffs modified from REVEAL and ESC/ERS Treatment Guidelines to include 4 strata (1=low, 2=intermediate-low, 3=intermediate-high, 4=high)
Risk category obtained by dividing sum of all grades by number of available variables and rounding to next integer
Suggested use
Baseline risk assessment
Follow-up risk assessment
Follow-up risk assessment
Overview
French retrospective analysis (2006-2016); N=1017
Swedish observational study (2008-2016); N=530
Variables
4 variables:
- WHO/NYHA FC
- 6MWD
- RAP
- CI*
8 variables:
- WHO FC
- 6MWD
- RAP
- CI
- NT-proBNP
- SvO2
- Pericardial effusion
- RA area
Risk calculation
Invasive: 4 variables above were used to classify patients by number of low-risk criteria present
Noninvasive: 3 variables (WHO/NYHA FC, 6MWD, and BNP/NT-proBNP) were used to classify patients by number of low-risk criteria present
Assigned risk-category grades to each variable based on ESC/ERS Treatment Guidelines (1=low, 2=intermediate, 3=high)
Risk category obtained by dividing sum of all grades by number of available variables and rounding to nearest integer
Suggested use
Follow-up risk assessment
Baseline risk assessment
Follow-up risk assessment