Pulmonary Hypertension in Pediatric Patients With Cystic Fibrosis During Acute Pulmonary Exacerbations: Prevalence and Associated Factors Publisher Pubmed



Roshanzamir Z ; Mohammadi F ; Shirzadi R
Source: BMC Pulmonary Medicine Published:2026

Abstract

Background: Cystic fibrosis (CF) is a multi-organ disorder in which respiratory complications account for the majority of its cause of mortality. This study aimed to investigate the factors associated with pulmonary hypertension (PH) in pediatric patients with CF and acute pulmonary exacerbations (PEx). Methods: This is a prospective cross-sectional study that enrolled children with CF who were hospitalized with PEx in a university hospital between 2020 and 2022. All patients underwent echocardiography, and their pulmonary artery pressure (PAP) was measured. They were then divided into two groups based on the presence or absence of PH. Clinical symptoms, spirometry, six-minute walk tests, laboratory findings, chest radiography, and other clinical parameters were compared in these two groups. The restricted cubic spline was plotted for variables with nonlinear associations with PH. Result: A total of 107 pediatric patients were included in this study. The prevalence of PH in the studied population was 24.3%. Group 1 consisted of 81 patients with normal PAP values (PAP < 25 mmHg), and group 2 included 26 patients with increased levels of PAP (PAP ≥ 25 mmHg). Group 2 had significantly higher median age, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) levels, as well as a greater frequency of major chest X-ray abnormalities and NIV use compared to group 1. Univariate logistic regression demonstrated that older age (OR 1.191, 95% CI 1.052–1.348, p = 0.006), elevated CRP (OR 1.027, 95% CI 1.009–1.046, p = 0.004), ESR ≥ 21 mm/hr (OR: 3.567, 95% CI: 1.350–9.427, p = 0.010), lower lymphocyte counts (OR 0.972, 95% CI 0.946–0.999, p = 0.044), and NIV requirement (OR 3.055, 95% CI 1.230–7.586, p = 0.016) were significantly associated with an increased likelihood of PH. In multivariate analyses adjusted for confounders, older age (OR 1.176, 95% CI 1.035–1.337, p = 0.013), elevated CRP (OR 1.024, 95% CI 1.004–1.044, p = 0.020), ESR ≥ 21 mm/hr (OR: 1.149, 95% CI: 1.008–1.310, p = 0.037), and NIV requirement (OR 2.860, 95% CI 1.102–7.422, p = 0.031) remained independently associated with having PH. Conclusion: In patients with CF and PEx, factors that suggest the possibility of concurrent PH include older age, infiltration or bronchiectasis on chest X-ray, NIV requirements, and elevated inflammatory markers. © The Author(s) 2025.