Heart Lung. 2026 Jan 8;78:102701. doi: 10.1016/j.hrtlng.2025.102701. Online ahead of print.
ABSTRACT
BACKGROUND: As survival improves for people with cystic fibrosis (PwCF) in the era of CFTR modulators, cardiovascular (CV) diseases are emerging as clinically important comorbidities. Beyond age-related risks, mechanistic pathways such as systemic inflammation, chronic hypoxia, CF-related diabetes, and CFTR-related endothelial dysfunction may contribute to CV injury. However, national-level data on CV outcomes in PwCF remain limited.
OBJECTIVES: We hypothesized that primary cardiac admissions in PwCF are increasing over time and associated with worse in-hospital outcomes compared to non-cardiac admissions.
METHODS: We retrospectively analyzed adult (≥18 years) PwCF hospitalizations in the U.S. National Inpatient Sample (2016-2022). Primary cardiac admissions were defined by a principal diagnosis of atrial fibrillation (AF), heart failure (HF), or myocardial infarction (MI) using ICD-10 codes. Outcomes included in-hospital mortality, length of stay (LOS), charges, and discharge disposition. Temporal trends in cardiac admissions were modeled using negative binomial regression with an offset for total CF hospitalizations; Joinpoint regression was performed as a complementary method. Descriptive statistics and multivariable regression models adjusted for age, sex, and race were used. A p-value <0.05 was considered statistically significant.
RESULTS: Among 121,290 PwCF hospitalizations, 520 (0.43%) were for cardiac causes. PwCF with cardiac admissions were older (median 62 vs. 29 years, p < 0.001) and had more traditional CV comorbidities. Cardiac admission rates increased by 16.4% per year from 2016 to 2022 (IRR 1.16 [1.04-1.29], p = 0.009) in negative binomial regression. Joinpoint regression detected no significant inflection points and estimated a non-significant APC of 16.4% per year (95% CI 10.9-57.4, p = 0.214). Unadjusted mortality was higher for cardiac vs. non-cardiac admissions (OR 3.70, 95% CI 1.61-8.53, p = 0.002), but not significant after adjustment (OR 1.36, 95% CI 0.55-3.34, p = 0.468).
CONCLUSION: Our findings indicated higher in-hospital mortality among PwCF admitted for cardiac causes, and more discharge to nursing facilities among PwCF admitted for cardiac causes. There is a need for greater CV screening, and geriatric care in PwCF.
PMID:41512346 | DOI:10.1016/j.hrtlng.2025.102701