Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).
BACKGROUND
Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use.


RESULTS
Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique.


CONCLUSIONS
We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.
Benza
Raymond L
RL
The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, PA 15212, USA. rbenza@wpahs.org
Miller
Dave P
DP
Gomberg-Maitland
Mardi
M
Frantz
Robert P
RP
Foreman
Aimee J
AJ
Coffey
Christopher S
CS
Frost
Adaani
A
Barst
Robyn J
RJ
Badesch
David B
DB
Elliott
C Gregory
CG
Liou
Theodore G
TG
McGoon
Michael D
MD
D000328:Adult; D000368:Aged; D001794:Blood Pressure; D002248:Carbon Monoxide / Q000097:blood; D018572:Disease-Free Survival; D005260:Female; D006339:Heart Rate; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011446:Prospective Studies; D012042:Registries; D051437:Renal Insufficiency / Q000097:blood / Q000401:mortality* / Q000503:physiopathology; D015996:Survival Rate; D014655:Vascular Resistance
D020097:Natriuretic Peptide, Brain; D002248:Carbon Monoxide