Abstract
BackgroundPulmonary artery (PA) enlargement, defined as pulmonary artery to ascending aorta diameter ratio (PA:A)>1 on computed tomography (CT), is a marker of pulmonary vascular disease in chronic lung diseases. PA enlargement is prevalent in cystic fibrosis (CF), but its relationship to hemodynamics and prognostic utility in severe CF are unknown. We hypothesized that the PA:A would have utility in identifying pulmonary hypertension (PH) in severe CF and that PA enlargement would be associated with reduced transplant-free survival.MethodsWe conducted a retrospective study of adults with CF undergoing lung transplant evaluation at a single center between 2000 and 2015. CT, right heart catheterization (RHC), and clinical data were collected. The PA:A was measured from a single CT slice. We measured associations between PA:A and invasive hemodynamic parameters including PH defined as a mPAP ≥25mmHg using adjusted linear and logistic regression models. Kaplan-Meier and adjusted Cox regression models were used to measure associations between PA:A>1, RHC-defined PH, and transplant-free survival in severe CF.ResultsWe analyzed 78 adults with CF that had CT scans available for review, including 44 that also had RHC. RHC-defined PH defined as a mPAP ≥25mmHg was present in 36% of patients with CF undergoing transplant evaluation. The PA:A correlated with mPAP (r = 0.73; 95% CI 3.87–7.80; p<0.001) and PVR (r = 0.42, p = 0.005) and the PA:A>1 was an independent predictor of PH (aOR 4.50; 95% CI 1.05–19.2; p = 0.042). PA:A>1 was independently associated with increased hazards for death or transplant (aHR 2.69; 95% CI 1.41–5.14; P = 0.003). The presence of mPAP ≥25mmHg was independently associated with decreased survival in this cohort.ConclusionsPA enlargement is associated with pulmonary hemodynamics and PH in severe CF. PA enlargement is an independent prognostic indicator of PH and decreased survival in this population.
Highlights
Cystic fibrosis (CF) is an autosomal recessive genetic disorder that leads to functional impairment in the cystic fibrosis transmembrane conductance regulator (CFTR) channel, leading to impaired mucociliary transport, recurrent infections, airflow obstruction, bronchiectasis, and altered gas exchange
The Pulmonary artery (PA):A correlated with mean pulmonary artery pressure (mPAP) (r = 0.73; 95% cardiac index (CI) 3.87–7.80; p1 was an independent predictor of pulmonary hypertension (PH)
PA enlargement is associated with pulmonary hemodynamics and PH in severe CF
Summary
Cystic fibrosis (CF) is an autosomal recessive genetic disorder that leads to functional impairment in the cystic fibrosis transmembrane conductance regulator (CFTR) channel, leading to impaired mucociliary transport, recurrent infections, airflow obstruction, bronchiectasis, and altered gas exchange. Lung function impairment is strongly associated with morbidity and mortality [1, 2]. Other comorbid conditions including pulmonary hypertension (PH) further negatively impact survival in CF. The presence of PH was shown to be a strong independent predictor of morbidity and mortality in adults with CF, with the risk of mortality higher among patients with CF compared with other patient populations awaiting lung transplant [4, 5]. Even subclinical PH can have a negative impact on exercise capacity, perceived dyspnea, quality of life, and mortality [6, 7]. We hypothesized that the PA:A would have utility in identifying pulmonary hypertension (PH) in severe CF and that PA enlargement would be associated with reduced transplant-free survival
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