Derivation of a Risk Score (REVEAL-ECHO) Based on Echocardiographic Parameters of Patients With Pulmonary Arterial Hypertension

El‐Kersh, Karim; Zhao, Carol; Elliott, Greg; Farber, Harrison W.; Gomberg‐Maitland, Mardi; Selej, Mona; Garcia-Ferrer, Josephine; Benza, Raymond L.

doi:10.1016/j.chest.2022.12.045

Cited by 13 publications

(9 citation statements)

References 27 publications

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“… 78 The REVEAL echocardiographic risk score was derived using retrospective echocardiographic data from the REVEAL registry database (RV chamber enlargement, reduced RV systolic function, tricuspid regurgitation severity, and pericardial effusion), and was shown to discriminate risk and signal probability of 12‐month survival. 79 However, these findings should be validated in larger, multicenter cohorts.…”

Section: Future Directionsmentioning

confidence: 96%

“…Another study also reported that the addition of surrogate markers of RV–pulmonary artery coupling (RV basal diameter, right atrial area/pressure, tricuspid regurgitation velocity, and TAPSE) to COMPERA and French risk stratification tools improved risk stratification 78 . The REVEAL echocardiographic risk score was derived using retrospective echocardiographic data from the REVEAL registry database (RV chamber enlargement, reduced RV systolic function, tricuspid regurgitation severity, and pericardial effusion), and was shown to discriminate risk and signal probability of 12‐month survival 79 . However, these findings should be validated in larger, multicenter cohorts.…”

Section: Future Directionsmentioning

confidence: 99%

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Evolution and optimization of clinical trial endpoints and design in pulmonary arterial hypertension

2023

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Selection of endpoints for clinical trials in pulmonary arterial hypertension (PAH) is challenging because of the small numbers of patients and the changing expectations of patients, clinicians, and regulators in this evolving therapy area. The most commonly used primary endpoint in PAH trials has been 6‐min walk distance (6MWD), leading to the approval of several targeted therapies. However, single surrogate endpoints such as 6MWD or hemodynamic parameters may not correlate with clinical outcomes. Composite endpoints of clinical worsening have been developed to reflect patients' overall condition more accurately, although there is no standard definition of worsening. Recently there has been a shift to composite endpoints assessing clinical improvement, and risk scores developed from registry data are increasingly being used. Biomarkers are another area of interest, although brain natriuretic peptide and its N‐terminal prohormone are the only markers used for risk assessment or as endpoints in PAH. A range of other genetic, metabolic, and immunologic markers is currently under investigation, along with conventional and novel imaging modalities. Patient‐reported outcomes are an increasingly important part of evaluating new therapies, and several PAH‐specific tools are now available. In the future, alternative statistical techniques and trial designs, such as patient enrichment strategies, will play a role in evaluating PAH‐targeted therapies. In addition, modern sequencing techniques, imaging analyses, and high‐dimensional statistical modeling/machine learning may reveal novel markers that can play a role in the diagnosis and monitoring of PAH.

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Section: Future Directionsmentioning

confidence: 96%

Section: Future Directionsmentioning

confidence: 99%

Evolution and optimization of clinical trial endpoints and design in pulmonary arterial hypertension

2023

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“…We calculated the REVEAL lite score as well as the recently described REVEAL-echo score for all participants. 23,27 Patients were followed for the primary outcome of time to death or transplantation within 5 years. Mortality and lung transplantation outcome were curated through chart review and national death index.…”

Section: Clinical Laboratory and Outcomes Datamentioning

confidence: 99%

“…Deriving these quantitative thresholds would not only be variable for consistency but would provide quantitative criteria for imaging-based risk scores such as the recently described REVEAL-ECHO. 23 The main objective of this study is to propose a consistent echocardiographic grading system in PAH anchored on physiological relationships and outcome analysis. Our first objective was to better quantify mathematical and physiological relationships between right heart variables in PAH.…”

Section: Introductionmentioning

confidence: 99%

Identifying consistent echocardiographic thresholds for risk stratification in pulmonary arterial hypertension

Celestin,

Bagherzadeh,

Ichimura

et al. 2024

Pulm. circ.

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Several indices of right heart remodeling and function have been associated with survival in pulmonary arterial hypertension (PAH). Outcome analysis and physiological relationships between variables may help develop a consistent grading system.Patients with Group 1 PAH followed at Stanford Hospital who underwent right heart catheterization and echocardiography within 2 weeks were considered for inclusion. Echocardiographic variables included tricuspid annular plane systolic excursion (TAPSE), right ventricular (RV) fractional area change (RVFAC), free wall strain (RVFWS), RV dimensions, and right atrial volumes. The main outcome consisted of death or lung transplantation at 5 years. Mathematical relationships between variables were determined using weighted linear regression and severity thresholds for were calibrated to a 20% 1‐year mortality risk.PAH patients (n = 223) had mean (SD) age of 48.1 (14.1) years, most were female (78%), with a mean pulmonary arterial pressure of 51.6 (13.8) mmHg and pulmonary vascular resistance index of 22.5(6.3) WU/m2. Measures of right heart size and function were strongly related to each other particularly RVFWS and RVFAC (R2 = 0.82, p < 0.001), whereas the relationship between TAPSE and RVFWS was weaker (R2 = 0.28, p < 0.001). Death or lung transplantation at 5 years occurred in 78 patients (35%). Guided by outcome analysis, we ascertained a uniform set of parameter thresholds for grading the severity of right heart adaptation in PAH. Using these quantitative thresholds, we, then, validated the recently reported REVEAL‐echo score (AUC 0.68, p < 0.001).This study proposes a consistent echocardiographic grading system for right heart adaptation in PAH guided by outcome analysis.

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“…Four parameters including right ventricular chamber enlargement, right ventricular reduced systolic function, tricuspid regurgitation severity, and pericardial effusion were combined into an echocardiographic risk score based on the REVEAL Registry. Incorporation of these echocardiographic parameters was found to be predictive of survival in PAH [ 32 ▪▪ ]. Given the importance of right ventricular function in mortality in pulmonary hypertension, incorporation of right ventricular assessment in risk stratification may continue to improve risk stratification tools in the future.…”

Section: Right Ventricular Function As a Goal For Pulmonary Arterial ...mentioning

confidence: 99%

Right ventricular assessment in pulmonary hypertension

Labrada

Vaidy

Vaidya

2023

Current Opinion in Pulmonary Medicine

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Purpose of review The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis. Recent findings The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme. Summary Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.

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Derivation of a Risk Score (REVEAL-ECHO) Based on Echocardiographic Parameters of Patients With Pulmonary Arterial Hypertension

Cited by 13 publications

References 27 publications

Evolution and optimization of clinical trial endpoints and design in pulmonary arterial hypertension

Evolution and optimization of clinical trial endpoints and design in pulmonary arterial hypertension

Identifying consistent echocardiographic thresholds for risk stratification in pulmonary arterial hypertension

Right ventricular assessment in pulmonary hypertension

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