Validating REVEAL: Can Scores Accurately Predict Survival in SSc-PAH? - The Cardiology Advisor

Posted: 03 Jun 2019 12:00 AM PDT

When predicting 1-year survival for patients with newly diagnosed systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH), REVEAL prediction scores should be interpreted with caution, particularly in patients with a higher predicted risk, according to research published in Arthritis and Rheumatology.

The recently developed Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry) prognostic equation and risk score calculator can be used to predict 1-year survival in patients with PAH. For this particular study, researchers sought to evaluate the predictive accuracy of the equation and risk score calculator in patients with incident SSc-PAH.

Patients participating in the validation cohort were drawn from 2 different cohorts: the Johns Hopkins Pulmonary Hypertension Program (JHPHP) Registry and the multicenter Pulmonary Hypertension Assessment and Recognition of Outcome in Scleroderma (PHAROS) Registry.

Patients from the JHPHP registry were consecutive patients with SSc, who were newly diagnosed with PAH via right heart catheterization between January 1, 2000 and July 1, 2015. Exclusions were applied to patients with evidence of obstructive or interstitial lung disease, rheumatologic overlap syndrome, previous treatment with active drugs for pulmonary hypertension, or inclusion in the initial REVEAL Registry.

Patients from the PHAROS Registry had established SSc and either a diagnosis of pulmonary hypertension within the previous 6 months or a classification of increased risk of developing PAH. Researchers noted that because Johns Hopkins is a PHAROS site, Johns Hopkins patients were removed from the PHAROS cohort because the JHPHP Registry included more complete patient information.

Investigators assessed 19 parameters and predicted the probability of 1-year survival for each patient. Patients were assigned points values and were then stratified into 5 risk groups: low (1-7 points), average (8 points), moderately high (9 points), high (10-11 points), and very high (≥12 points).

In total, the validation cohort included 292 patients (n = 117 from JHPHO and n = 176 from PHAROS); patients were primarily white women, age ≥60 years, with World Health Organization Functional Class II or III; however, patients in the JHPHP cohort were younger and were more likely to present with limited SSc, worse functional class, shorter distance on 6-minute walk test, lower reduced lung diffusion capacity, and worse hemodynamics.

Mean REVEAL risk score was higher in patients in the JHPHP Registry vs the PHAROS registry (9±2 vs 8±2; P =.005), although researchers noted that the PHAROS Registry does not record several data points recorded by the JHPHP Registry, so patients could not receive those points.

In total, 36 patients died and overall survival was 87.4% (95% CI, 82.9%-90.7%). C-indices for the REVEAL prognostic equation (0.73; 95% CI, 0.65-0.82) and risk score calculator (0.74, 95% CI: 0.66-0.82, respectively) showed "good discrimination," according to investigators. Slope of calibration was 0.71 (95% CI, 0.4-1.01), indicating a marginal overall fit.

According to hazard ratios, researchers found that patients classified as high risk had the worst survival.

"This suggests that while overall discrimination was good, discrimination for those at higher risk may not be as accurate," the researchers noted.

One important limitation of the study included concerns with missing data, including right atrial pressure, heart rate, and systolic blood pressure in the PHAROS cohort. Because these variables are included with increased risks, their absence "may have biased predictions toward a lower predicted mortality in the PHAROS cohort," according to the researchers.

"Our results show that when applied to a cohort of patients with newly diagnosed SSc-PAH, the REVEAL prognostic equation and risk score offer similar measures of overall discrimination as they did in the original model development cohort," the researchers wrote. "However, discrimination and calibration appear to be less accurate in patients in the highest risk groups, indicating that the REVEAL prediction model may not perform adequately in separating and predicting survival in newly diagnosed [patients with SSc-PAH] with high risk features and the lowest predicted probabilities of 1-year survival."

Reference

Mullin CJ, Khair RM, Damico RL, et al; PHAROS investigators. Validation of the REVEAL prognostic equation and risk score calculator in incident systemic sclerosis-associated pulmonary arterial hypertension [published online May 8, 2019]. Arthritis Rheumatol. doi:10.1002/art.40918

This article originally appeared on Rheumatology Advisor

Topics:

Pulmonary Arterial Hypertension Pulmonary Hypertension

Pulmonary Hypertension Survival Predicted by Cardiopulmonary Reserve Index - Pulmonology Advisor

Posted: 22 May 2019 12:00 AM PDT

This article is part of Pulmonology Advisor's coverage of the American Thoracic Society International Conference, taking place in Dallas, Texas. Our staff will report on medical research related to asthma and other respiratory conditions, conducted by experts in the field. Check back regularly for more news from ATS 2019.

DALLAS — The cardiopulmonary reserve index (CPRI) may be an effective predictor of survival in patients with pulmonary hypertension (PH), particularly in patients with PH caused by left heart disease (LHD), according to study findings presented at the American Thoracic Society International Conference held May 17-22, in Dallas, Texas.

"The [cardiovascular reserve index] has been proposed as an estimate of cardiovascular reserve based on stroke volume, systemic vascular resistance, respiratory rate, and body surface area in various conditions including heart failure and shock," researchers wrote. Therefore, they sought to adapt the cardiovascular reserve index formula to pulmonary circulation to determine whether it could predict survival in patients with PH.

The researchers retrospectively obtained data from a total of 4363 patients who underwent a first diagnostic right heart catheterization between 1996 and 2006. A total of 1710 patients were diagnosed with PH; 125 cases of pulmonary arterial hypertension, 1410 cases of PH-LHD, 26 cases with PH caused by lung diseases and/or hypoxia, 105 cases with chronic thromboembolic PH, and 1 case with PH from unclear/multifactorial mechanisms. The following simplified formula was used to calculate CPRI: (20*mean pulmonary arterial pressure—mean pulmonary arterial wedge pressure)/(heart rate*body surface area).

In the overall PH group, CPRI was a predictor of survival according to the Cox regression analysis (hazard ratio [HR], 2.105; 95% CI, 1.319-3.360; P =.002). In a subgroup analysis stratified by PH groups, CPRI was only predictive of survival in patients with PH-LHD (HR, 4.370; 95% CI, 2.109-9.054; P <.001). The inclusion of pulmonary vascular resistance (HR, 1.017; 95% CI, 0.958-1.079; P =.587) in a multivariate Cox regression model demonstrated that CPRI was a persistent predictor of prognosis (HR, 3.640; 95% CI, 1.017-14.653; P =.046). Both CPRI (HR, 12.190; 95% CI, 4.310-34.478; P <.001) and diastolic pulmonary vascular pressure gradient (HR, 0.979; 95% CI, 0.936-0.995; P =.001) were considered multivariable predictors of PH-LHD prognosis.

The researchers noted that further studies are needed to determine why CPRI is only a predictor of survival in PH-LHD and whether it could be helpful in guiding treatment.

Reference

Shafran I, Gerges C, Gerges M, Segel M, Lang IM. Cardiopulmonary reserve index as predictor of survival in patients with pulmonary hypertension due to left heart disease. Presented at: the American Thoracic Society International Conference; May 22, 2019; Dallas, TX. Abstract A6794/P995.

Topics:

ATS 2019 Pulmonary Hypertension

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