Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension.
BACKGROUND
Current management guidelines for pulmonary arterial hypertension (PAH) recommend a treatment choice based primarily on World Health Organization (WHO) functional class. This study was designed to assess how the incorporation of readily obtained clinical and test-based information may significantly improve the prediction of outcomes over functional class alone.


METHODS
Clinical and hemodynamic variables were assessed in 484 consecutive patients presenting with WHO group 1 PAH. The primary outcome measure was time to all-cause mortality over 5 years from the index presentation (data available in all). Follow-up was censored at the time of lung or heart/lung transplant in 21 patients or at 5 years. Predictors of mortality were assessed sequentially using Cox models, with the step-wise incorporation of clinical variables, echocardiographic, and catheterization findings. Results were further compared with the REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) prediction score.


RESULTS
Overall median survival was 237 weeks (95% CI, 196-266), corresponding to 1-year, 3-year, and 5-year survival rates of 81.1% (77.0, 84.7), 61.1% (56.5, 65.3), and 47.9% (43.2, 52.4), respectively. The prediction of mortality was improved incrementally by incorporating clinical and echocardiographic measures with a concordance index (c-index) of 0.84 compared with that of 0.60 with functional class alone. The REVEAL prediction score was validated independently in this cohort to predict both 1-year and 5-year mortality. It had a prediction c-index of 0.71.


CONCLUSIONS
The integration of routine PAH clinical (predominantly noninvasive) parameters predicts long-term outcome better than functional class and, hence, should be incorporated into medical management decisions.
Pulmonary Hypertension Clinic, Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN. Electronic address: kane.garvan@mayo.edu.
Garvan C
GC
Kane
Department of Health Sciences Research, Mayo Clinic, Rochester, MN.
Hilal
H
Maradit-Kremers
Department of Health Sciences Research, Mayo Clinic, Rochester, MN.
Josh P
JP
Slusser
Department of Health Sciences Research, Mayo Clinic, Rochester, MN.
Chris G
CG
Scott
Pulmonary Hypertension Clinic, Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN.
Robert P
RP
Frantz
Pulmonary Hypertension Clinic, Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, MN.
Michael D
MD
McGoon
D000328:Adult; D000368:Aged; D016275:Atrial Function; D001794:Blood Pressure; D015331:Cohort Studies; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000401:mortality* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D015996:Survival Rate; D014655:Vascular Resistance; D016276:Ventricular Function