PAH Is a Progressive Disease, Even for Mildly Symptomatic Patients1,2

Patients With Pulmonary Arterial Hypertension (PAH) Can Be at Greater Risk Than They Appear: Findings From the REVEAL Registry1,2*

1 in 4

(24% [58/244]) occurred in newly diagnosed patients in FC II1
More than
of PAH-related

(56% [136/244]) occurred in newly diagnosed patients in FC III1

Gestalt Assessment May Underestimate Patient Risk3

In a survey,
of treatment decision-makers

(50/121) reported not using a structured tool to assess risk in patients with PAH4

of these decision-makers

who did not use a structured tool to assess risk in patients with PAH reported that they relied on gestalt4

A retrospective chart analysis of patients considered low risk by gestalt showed that:
11% TO 36%
were categorized as
intermediate risk

using various structured risk assessment tools (n=63 and 47, respectively)
4% TO 28%
were categorized as high risk
using various structured risk assessment tools (n=46 and 47, respectively)

Risk Assessment Is Key in Patients With PAH5

With gestalt alone, patient risk in PAH may be underestimated3

Structured risk assessments are important for assessing your patients diagnosed with PAH. Comprehensive assessment helps determine patient prognosis, monitor disease progression and response to treatment, and impact treatment decisions. If widely integrated, risk assessment may help enhance consistency and timeliness of care, allowing for optimal care that may ultimately improve mortality while reducing morbidity in patients with PAH.5,6

The 2015 ESC/ERS Guidelines recommend a comprehensive approach to risk assessment as there is no single variable that can provide sufficient diagnostic and prognostic information. Comprehensive, multiparameter risk assessment tools include REVEAL 2.0 and REVEAL Lite 2.5,7,8

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*REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) was a US-based, observational registry involving 55 academic- and community-based treatment centers. 3515 patients enrolled between March 2006 and December 2009. REVEAL was funded and sponsored by Actelion Pharmaceuticals US, Inc.9
Newly diagnosed patients defined as having diagnostic RHC within 90 days of enrollment. Analysis evaluated 862 newly diagnosed patients for first-time hospitalization. Hospitalizations were categorized as PAH related or PAH unrelated based on case report forms. Categories were defined prior to independent review. Of the 862 patients, 257 were hospitalized for PAH, of which 244 patients had available FC assessment prior to hospitalization. Data shown represent patients with a PAH-related hospitalization and FC assessment.1
One hundred twenty-one PAH treatment decision-makers (physicians, nurse practitioners, and physician assistants) in the US were surveyed between February 7 and March 10, 2019 on their utilization of structured risk assessment tools in PAH management in clinical practice settings. Respondents could report using only clinical gestalt for risk assessment or choose from a number of structured risk assessment tools, including the REVEAL risk calculator, the French Pulmonary Hypertension Registry (FPHR) risk scoring system, the European Society of Cardiology (ESC)/European Respiratory Society (ERS) Guidelines for risk assessment, Comparative Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) risk stratification, the Swedish Pulmonary Arterial Hypertension Registry (SPAHR) risk assessment, or another tool (unspecified). Medical writing support was funded and sponsored by Actelion Pharmaceuticals Ltd.4
§Medical charts of 153 patients with FC II PAH were retrospectively analyzed to compare the results of risk assessment by clinical gestalt to those using structured risk assessment tools. COMPERA risk assessment classified 11% (n=63) of patients considered low risk by gestalt as intermediate risk. Using modified noninvasive FPHR, 36% (n=47) of patients considered low risk by gestalt were categorized as intermediate risk and 28% (n=47) as high risk. REVEAL 2.0 risk assessment classified 20% (n=46) of patients considered low risk by gestalt as intermediate risk and 4% (n=46) as high risk.3
FC=Functional Class; RHC=right heart catheterization.
References: 1. Burger CD, Long PK, Shah MR, et al. Characterization of first-time hospitalizations in patients with newly diagnosed pulmonary arterial hypertension in the REVEAL Registry. CHEST. 2014;146(5):1263-1273. 2. Frost AE, Badesch DB, Miller DP, et al. Evaluation of the predictive value of a clinical worsening definition using 2-year outcomes in patients with pulmonary arterial hypertension: a REVEAL Registry analysis. CHEST. 2013;144(5):1521-1529. 3. Sahay S, Tonelli AR, Selej M, et al. Risk assessment in patients with functional class II pulmonary arterial hypertension: comparison of physician gestalt with ESC/ERS and the REVEAL 2.0 risk score. PLoS One. 2020;15(11):e0241504. doi:10.1371/journal.pone.0241504 4. Wilson M, Keeley J, Kingman M, et al. Current clinical utilization of risk assessment tools in pulmonary arterial hypertension: a descriptive survey of facilitation strategies, patterns, and barriers to use in the United States. Pulm Circ. 2020;10(3):2045894020950186 and suppl. doi:10.1177/2045894020950186 5. Raina A, Humbert M. Risk assessment in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):390-398. 6. Hoffman KK, Benza RL, Kanwar M. Risk stratification in PAH. American College of Cardiology. Published January 31, 2019. Accessed August 25, 2021. 7. Galiè N, Humbert M, Vachiéry J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2015;46(4):903-975. 8. Benza RL, Kanwar MK, Raina A, et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension. CHEST. 2021;159(1):337-346. 9. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21(123):8-18.