Pulmonary Hypertension in Cystic Fibrosis with Advanced Lung Disease

• DOI: 10.1164/rccm.201407-1382oc
• Publication Date: 2014-09-15T18:23:32Z
• Authors: Don Hayes; Joseph D. Tobias; Heidi M. Mansour; Stephen Kirkby; Karen S. McCoy; Curt J. Daniels; Bryan A. Whitson
• Keywords: Univariate analysis

The impact of pulmonary hypertension (PH) on survival in cystic fibrosis (CF) remains unclear.To determine the influence PH CF population.The United Network for Organ Sharing database was queried from 1987 to 2013 identify first-time lung transplant candidates who were tracked wait list entry date until death or censoring PH. Using right heart catheterization measurements, mild defined as mean artery pressure greater than equal 25 mm Hg and severe 35 Hg.Of 2,781 patients, 2,100 used univariate analysis, 764 Kaplan-Meier function, 687 multivariate Cox models, 576 132 matching propensity PH, respectively. Univariate analysis found significant differences (hazard ratio [HR], 1.747; 95% confidence interval [CI], 1.387-2.201; P < 0.001) (HR, 2.299; CI, 1.639-3.225; 0.001). Further assessment by models identified risk associated with 1.757; 1.367-2.258; 2.284; 1.596-3.268; regression stratified matched pairs cases control subjects confirmed 1.919; 1.290-2.85; = 4.167; 1.709-10.157; 0.002).The manifestation is significantly increased patients advanced disease.