Validation of the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) pulmonary hypertension prediction model in a unique population and utility in the prediction of long-term survival

Cogswell, Rebecca; Kobashigawa, E.; McGlothlin, Dana; Shaw, Robin M.; Marco, Teresa De

doi:10.1016/j.healun.2012.08.009

Cited by 32 publications

(15 citation statements)

References 19 publications

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“…The calibration slope, or regression coefficient of the prognostic index (Z’βγ), was 0.707 (95% CI 0.400–1.014), and the test for a coefficient of 1 resulting in a P value of 0.06 indicated a borderline, but acceptable model fit for the prognostic equation. The C‐indexes for both the prognostic equation and the risk score are presented in Table , along with C‐indexes in the REVEAL cohorts and 2 other studies that evaluated REVEAL model performance . In predicting 1‐year survival in patients newly diagnosed as having SSc‐PAH, these estimates with the 2 REVEAL survival prediction tools indicated very good discrimination, and demonstrated that the C‐indexes in our validation cohort were similar to those in the REVEAL cohorts and the other validation studies.…”

Section: Resultssupporting

confidence: 65%

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Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Mullin

Khair

Damico

et al. 2019

Arthritis & Rheumatology

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on behalf of the PHAROS InvestigatorsObjective. A prognostic equation and risk score calculator derived from the Registry to Evaluate Early and Longterm Pulmonary Arterial Hypertension Disease Management (REVEAL) are being used to predict 1-year survival in patients with pulmonary arterial hypertension (PAH), but little is known about the performance of these REVEAL survival prediction tools in systemic sclerosis (SSc)-associated PAH (SSc-PAH).Methods. Prospectively gathered data from the Johns Hopkins Pulmonary Hypertension Program and Pulmonary Hypertension Assessment and Recognition of Outcome in Scleroderma Registries were used to evaluate the predictive accuracy of the REVEAL models for predicting the probability of 1-year survival in patients with SSc-PAH. Model discrimination was assessed by comparison of the Harrell's C-index, model fit was assessed using multivariable regression techniques, and model calibration was assessed by comparing predicted to observed survival estimates.Results. The validation cohort consisted of 292 SSc-PAH patients with a 1-year survival rate of 87.4%. The Cindex for predictive accuracy of the REVEAL prognostic equation (0.734, 95% confidence interval [95% CI] 0.652-0.816) and for the risk score (0.743, 95% CI 0.663-0.823) demonstrated good discrimination, comparable to that in the model development cohort. The calibration slope was 0.707 (95% CI 0.400-1.014), indicating that the overall model fit was marginal (P = 0.06). The magnitude of risk assigned to low distance on the 6-minute walk test (6MWD) and elevated serum levels of brain natriuretic peptide (BNP) was lower in the validation cohort than was originally seen in the REVEAL derivation cohort. Model calibration was poor, particularly for the highest risk groups.Conclusion. In predicting 1-year survival in patients newly diagnosed as having SSc-PAH, the REVEAL prognostic equation and risk score provide very good discrimination but poor calibration. REVEAL prediction scores should be interpreted with caution in newly diagnosed SSc-PAH patients, particularly those with higher predicted risk of poor 1-year survival resulting from a low 6MWD or a high BNP serum level.

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Section: Resultssupporting

confidence: 65%

“…In this combined REVEAL Registry cohort, only 500 patients had SSc‐PAH (15.5% of the combined cohort), of whom 166 were newly diagnosed as having PAH . Subsequent studies that have validated the REVEAL models have involved similarly small proportions of SSc‐PAH patients in their validation cohorts .…”

Section: Introductionmentioning

confidence: 99%

Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Mullin

Khair

Damico

et al. 2019

Arthritis & Rheumatology

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“…Each of these equations has been statistically validated in a population from the respective registry that did not contribute to construction of the model. The results of these analyses suggested an improvement in the survival of patients with PAH compared with that reported at the time of the US National Institutes of Health primary pulmonary hypertension registry [13,14,18,[22][23][24].…”

Section: Introductionmentioning

confidence: 76%

Validation of two predictive models for survival in pulmonary arterial hypertension

et al. 2015

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The French Pulmonary Hypertension Network (FPHN) registry and the Registry to Evaluate Early And Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) have developed predictive models for survival in pulmonary arterial hypertension (PAH). In this collaboration, we assess the external validity (or generalisability) of the FPHN ItinérAIR-HTAP predictive equation and the REVEAL risk score calculator.Validation cohorts approximated the eligibility criteria defined for each model. The REVEAL cohort comprised 292 treatment-naïve, adult patients diagnosed <1 year prior to enrolment with idiopathic, familial or anorexigen-induced PAH. The FPHN cohort comprised 1737 patients with group 1 PAH.Application of FPHN parameters to REVEAL and REVEAL risk scores to FPHN demonstrated estimated hazard ratios that were consistent between studies and had high probabilities of concordance (hazard ratios of 0.72, 95% CI 0. and 0.73, respectively).The REVEAL risk score calculator and FPHN ItinérAIR-HTAP predictive equation showed good discrimination and calibration for prediction of survival in the FPHN and REVEAL cohorts, respectively, suggesting prognostic generalisability in geographically different PAH populations. Once prospectively validated, these may become valuable tools in clinical practice. @ERSpublications REVEAL risk score and ItinérAIR-HTAP equation show geographic validity (generalisability) in distinct PAH populations http://ow.ly/INyx8

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“…The drugs most commonly associated with PAH are anorectics and abused drugs such as cocaine (6,7) and methamphetamine (8), abused stimulant drugs, and the most commonly prescribed anorectics, are all selective serotonin reuptake inhibitors (SSRIs) (9). Rothman was first to demonstrate, in 1999 that, like other drugs known to cause pulmonary hypertension, aminorex was a serotonin transporter substrate (10), although recent studies have raised questions as to whether the relationship between SSRI antidepressants and PAH is actually causal.…”

Section: Contextmentioning

confidence: 99%

Levamisole, Aminorex, and Pulmonary Arterial Hypertension: A Review

Karch¹,

Vaiano²,

Bertol³

2015

Razavi Int J Med

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Context: An epidemic of aminorex-associated pulmonary arterial hypertension (PAH), resulting in multiple fatalities, occurred in the 1970s ending only after the drug was removed from the market. In 2009, when it was found that horses de-wormed with the anthelmintic levamisole, metabolized that drug to aminorex, the same drug that caused the 1970s outbreak of PAH. The discovery would not have been cause for concern except to horseracing enthusiasts, who feared horse doping. However, at about the same time the relationship between levamisole and aminorex was discovered, cocaine cartels began adulterating cocaine with levamisole. The rationale for adulterating cocaine with levamisole remains to be established. The purpose of this short review article is to discuss possible reasons for levamisole contamination, new discoveries about the human pharmacokinetics of aminorex, and the possible relationship between aminorex and pulmonary arterial hypertension in man. Evidence acquisition: Medline contents, as well as estimates published by the U.S. drug enforcement agency (DEA), US state department, and the European monitoring center for drugs and drug abuse (EMCDDA) were reviewed and relevant articles retrieved. There is uniform agreement among the various monitoring agencies analyzed.Results: Approximately 70 percent of the U.S. cocaine supply and 40 percent of the European cocaine supply is contaminated with levamisole. Aminorex has the same binding affinities for serotonin and other neurotransmitters as amphetamine. As serotonin is considered to be an important factor in the development of PAH, the possibility of another epidemic of aminorex-induced PAH, this time among cocaine abusers, seems real, and threatening. Conclusions:The results of the first human pharmacokinetic studies of aminorex were first characterized in 2013. The results suggest that while humans could produce aminorex from levamisole, they probably do not convert very much. Even though cases of PAH have been reported in cocaine users, the latest pharmacokinetic studies suggest that very little aminorex is actually produced from the ingested levamisole, probably not enough to cause PAH. However, since both cocaine and aminorex can cause PAH the situation remains unclear. the lack of a clear answer is partially the development of an insidious pulmonary hypertension, producing subtle and non-specific symptoms in its early stages.

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Validation of the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) pulmonary hypertension prediction model in a unique population and utility in the prediction of long-term survival

Cited by 32 publications

References 19 publications

Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Validation of the REVEAL Prognostic Equation and Risk Score Calculator in Incident Systemic Sclerosis–Associated Pulmonary Arterial Hypertension

Validation of two predictive models for survival in pulmonary arterial hypertension

Levamisole, Aminorex, and Pulmonary Arterial Hypertension: A Review

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