Correlation Between Computed Tomography Expression of Pulmonary Hypertension and Severity of Lung Disease in Cystic Fibrosis Patients

Purpose:Pulmonary hypertension (PHTN) is a significant cause of morbidity and mortality in patients with cystic fibrosis (CF), but a routine noninvasive test for evaluating PHTN is lacking. We aimed to determine whether there is a correlation between computed tomography (CT) signs for PHTN and lung disease severity in CF.Materials and Methods:We retrospectively evaluated high-resolution CT studies of 91 consecutive CF patients. The ratios of the widest diameters of the main pulmonary artery (MPA), right pulmonary artery, and left pulmonary artery to the ascending aorta (Ao) were calculated. MPA/Ao>1 was considered indicative for PHTN. Lung severity was assessed with modified Brody scores for the left lung (LL), right lung (RL), and overall [Total Brody Score (TBS)]. The nonparametric Pearson (2) test was performed for determining the correlation between Brody score and objective CT signs for PHTN.Results:This study included 54 male and 37 female patients aged between 3 and 67 years (mean 17.6 y). Mean RL, LL, and TBS were 23.71, 23.31, and 47.15, respectively. Of the 91 patients, 28 (30.8%) had MPA/Ao>1, indicating a high probability for PHTN. Among these patients, the mean TBS was 38.8, as compared to mean of 50.8 in 63 patients with MPA/Ao1. No correlation was found for the RL and LL separately.Conclusion:There was no correlation between parenchymal lung disease severity assessed by the modified Brody score and CT signs of PHTN in CF patients.