Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension

Chest. 2011 Jun;139(6):1285-1293. doi: 10.1378/chest.10-1293. Epub 2010 Nov 11.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Atrial Function
  • Blood Pressure
  • Cohort Studies
  • Familial Primary Pulmonary Hypertension
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / physiopathology*
  • Male
  • Middle Aged
  • Outcome Assessment, Health Care
  • Predictive Value of Tests
  • Proportional Hazards Models
  • Survival Rate
  • Vascular Resistance
  • Ventricular Function