Fig 1.
Pulmonary arterial enlargement in severe cystic fibrosis.
A representative axial image of a CT scan at the level of the pulmonary artery (PA) bifurcation. The diameter of the PA is measured at the location indicated by the blue arrow. The diameter of the ascending aorta (A) is calculated using two perpendicular measurements indicated by the red arrows. PA enlargement (PA:A>1) is present in this image.
Fig 2.
CF = cystic fibrosis. CT = contrast tomography. RHC = right heart catheterization.
Table 1.
Baseline characteristics.
Fig 3.
Associations between PA enlargement and pulmonary hypertension measured at right-heart catheterization in cystic fibrosis. PA enlargement was associated with PH defined by mPAP ≥25 mmHg (*P<0.05) in logistic regression models adjusted for age, sex, FEV1 percent predicted, and supplemental oxygen use.
Abbreviations: CI = confidence interval; FEV1 = forced expiratory volume in 1-second; PA:A = pulmonary artery diameter to aorta diameter ratio.
Fig 4.
Kaplan-Meier curves for transplant-free survival in severe cystic fibrosis.
Survival according to the presence or absence of A) PA:A>1; or B) mPAP ≥25mmHg. The numbers of subjects at risk are displayed below each figure panel. Abbreviations: mPAP = mean pulmonary artery pressure; PA:A = pulmonary artery to ascending aorta diameter ratio; PH = pulmonary hypertension.