Abstract Background Cystic fibrosis (CF) is a heterogeneous monogenetic autosomal recessive inherited multisystem-disease that shortens life of affected patients. Overall, more than 80,000 people are afflicted by CF worldwide. Due to the progress regarding treatments during the last three decades, the long-term prognosis of patients with CF has enormously improved. However, individuals with CF are afflicted by an increased risk of venous throm-boembolism (VTE) due to chronic inflammation, use of central venous catheters, as well as acquired thrombophilia. VTE with its manifestations pulmonary embolism (PE) and deep vein thrombosis is accompanied by high morbidity and mortality. Purpose Since PE is a cardiovascular as well as pulmonary disease-manifestation caused by embolus material blocking the pulmonary artery bed, it is of interest whether CF as disease with focus on the lungs, but also affecting cardiovascular system, might influence cardiac deviations caused by PE and the prognosis of acute PE. Methods The German nationwide inpatient sample was used for this study (source: RDC of the Federal Statistical Office and the Statistical Offices of the federal states, DRG Statistics 2005-2020, own calculations). 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 72.0years, 53.0% females) in Germany were included in our study 2005-2020. Among these, a small minority of 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, P0.001) with 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, P0.001) compared to those without CF. In contrast, diabetes mellitus was more frequently detected in PE patients with CF (49.2% vs. 18.7%, P0.001). CF was independently of age, sex and comorbidities associated with higher case-fatality (OR 2.451 95%CI 1.542-3.896, P0.001), 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, P0.001). Conclusions PE patients with CF were younger at the PE event and accompanied by typical comorbidities such as diabetes and renal disease compared to PE patients without CF. CF was afflicted by higher complication rates comprising pneumonia, bleeding events and 2.5-fold increased case-fatality.
Keller et al. (Sat,) studied this question.