Cystic fibrosis and its impact on prognosis in pulmonary embolism

Abstract

Background Pulmonary embolism (PE) and cystic fibrosis (CF) are both diseases that impact the cardiovascular-pulmonary system. While CF is a rare heterogeneous monogenetic autosomal-recessive inherited multisystem-disease that reduces life-expectancy, PE is a common emergency event with also high morbidity and mortality. We aimed to investigate the impact of CF on prognosis of PE patients. Methods The German nationwide inpatient sample was used for this study. All patient-cases of patients with PE in Germany 2005–2020 were included and stratified for CF. Results Overall, 1,373,084 patient-cases of PE patients (median age 72.0 years, 53.0 % females) in Germany were included in our study 2005–2020. Among these, 126 patients (0.01 %) were coded with CF. PE patients with CF were younger (33.0 [24.0–43.0] vs. 72.0 [60.0–80.0] years, P < 0.001) displaying a lower prevalence of arterial hypertension, hyperlipidemia and lower comorbidity-burden (Charlson comorbidity index: 2.0 [1.0–4.0] vs. 4.0 [3.0–7.0], P < 0.001) compared to those without CF. In contrast, diabetes mellitus was more frequent in PE patients with CF (49.2 % vs. 18.7 %, P < 0.001). After adjustment for age, sex and comorbidities, CF was associated with higher case-fatality (OR 2.451 [95 % CI 1.542–3.896], P < 0.001), pneumonia (OR 1.518 [95 % CI 1.059–2.176], P = 0.023) and necessity regarding transfusion of blood constituents (OR 3.702 [95 % CI 2.540–5.396], P < 0.001). Conclusions PE patients with CF were younger at the time of PE event and accompanied by typical comorbidities such as diabetes mellitus and renal disease compared to PE patients without CF. CF was associated with higher complication rates comprising pneumonia, bleeding events and 2.5-fold increased case-fatality.