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.
BACKGROUND
The Registry to Evaluate Early and Long-Term Pulmonary Arterial (PAH) Hypertension Disease Management (REVEAL) model was designed to predict 1-year survival in patients with PAH. Multivariate prediction models need to be evaluated in cohorts distinct from the derivation set to determine external validity. In addition, limited data exist on the utility of this model in the prediction of long-term survival.


METHODS
REVEAL model performance was assessed to predict 1-year and 5-year outcomes, defined as survival or composite survival or freedom from lung transplant, in 140 patients with PAH.


RESULTS
The validation cohort had a higher proportion of human immunodeficiency virus (7.9% vs 1.9%, p < 0.0001), methamphetamine use (19.3% vs 4.9%, p < 0.0001), and portal hypertension PAH (16.4% vs 5.1%, p < 0.0001) compared with the development cohort. The C-index of the model to predict survival was 0.765 at 1 year and 0.712 at 5 years of follow-up. The C-index of the model to predict composite survival or freedom from lung transplant was 0.805 and 0.724 at 1 and 5 years of follow-up, respectively. Prediction by the model, however, was weakest among patients with intermediate-risk predicted survival.


CONCLUSIONS
The REVEAL model had adequate discrimination to predict 1-year survival in this small but clinically distinct validation cohort. Although the model also had predictive ability out to 5 years, prediction was limited among patients of intermediate risk, suggesting our prediction methods can still be improved.
Division of Cardiology, University of California, San Francisco, San Francisco, CA, USA. rebecca.cogswell@ucsf.edu
Rebecca
R
Cogswell
Erin
E
Kobashigawa
Dana
D
McGlothlin
Robin
R
Shaw
Teresa
T
De Marco
D000328:Adult; D000368:Aged; D015897:Comorbidity; D019468:Disease Management; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D015658:HIV Infections / Q000453:epidemiology; D006801:Humans; D006975:Hypertension, Portal / Q000453:epidemiology; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000401:mortality* / Q000628:therapy*; D016040:Lung Transplantation; D008297:Male; D008694:Methamphetamine; D008875:Middle Aged; D015233:Models, Statistical; D012042:Registries; D012189:Retrospective Studies; D019966:Substance-Related Disorders / Q000453:epidemiology; D015996:Survival Rate; D013997:Time Factors
D008694:Methamphetamine