Liverpool Centre for Cardiovascular Science, University of Liverpool, Liverpool John Moores University, and Liverpool Heart and Chest Hospital, Liverpool, UK
Adult Cystic Fibrosis Centre, Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, UK.
Eur Respir J. 2023 Oct 26;62(4). doi: 10.1183/13993003.00174-2023. Print 2023 Oct.
Although people living with cystic fibrosis (PwCF) often have some risk factors for cardiovascular disease, including diabetes and chronic inflammation, little is known about the long-term cardiac risk in this condition. We aimed to determine the characteristics, rates and outcomes for cardiac disease in CF.
We looked at rates and outcomes for cardiac disease in 5649 adult PwCF in the UK CF Registry and 6265 adult PwCF in TriNetX (a global federated database of electronic healthcare record data). We used propensity matching to compare risk of major adverse cardiac events (MACE) (myocardial infarction, left-sided heart failure and atrial fibrillation) in PwCF against matched non-CF comparators in the general population and other inflammatory diseases.
PwCF had a high prevalence of diabetes but low rates of hypertension and obesity. Some cardiac risk factors (age, diabetes and hypertension) were associated with MACE, but relationships between disease-specific risk factors (lung function and intravenous antibiotic days) were also observed. In propensity score-matched analyses, PwCF had higher risk of MACE than matched general population comparators (hazard ratio (HR) 1.65, 95% CI 1.40-1.95; p<0.001) and an equivalent or higher relative risk compared with other inflammatory conditions considered "high risk" for cardiovascular disease, including systemic lupus erythematosus (HR 0.95, 95% CI 0.82-1.09; p=0.44), rheumatoid arthritis (HR 1.21, 95% CI 1.00-1.48; p<0.001) and HIV (HR 0.93, 95% CI 0.82-1.06; p=0.29).
PwCF are at increased risk of adverse cardiac disease events. Future work should focus on defining determinants of cardiovascular risk such that appropriate risk stratification can be employed.
尽管囊性纤维化（CF）患者常存在一些心血管疾病的风险因素，包括糖尿病和慢性炎症，但对于 CF 患者的长期心脏风险知之甚少。本研究旨在明确 CF 患者的心脏疾病特征、发生率和结局。
我们观察了英国 CF 注册处的 5649 例成年 CF 患者和 TriNetX（一个全球电子医疗记录数据联邦数据库）的 6265 例成年 CF 患者的心脏疾病发生率和结局。我们采用倾向评分匹配比较 CF 患者与普通人群和其他炎症性疾病中的非 CF 对照者的主要不良心脏事件（MACE）（心肌梗死、左侧心力衰竭和心房颤动）风险。
CF 患者的糖尿病患病率较高，但高血压和肥胖的发病率较低。一些心脏危险因素（年龄、糖尿病和高血压）与 MACE 相关，但也观察到疾病特异性危险因素（肺功能和静脉用抗生素天数）之间的关系。在倾向评分匹配分析中，CF 患者的 MACE 风险高于匹配的普通人群对照者（风险比（HR）1.65，95%CI 1.40-1.95；p<0.001），与其他被认为心血管疾病“高危”的炎症性疾病（包括系统性红斑狼疮（HR 0.95，95%CI 0.82-1.09；p=0.44）、类风湿关节炎（HR 1.21，95%CI 1.00-1.48；p<0.001）和 HIV（HR 0.93，95%CI 0.82-1.06；p=0.29）相比，风险比相等或更高。
CF 患者发生不良心脏疾病事件的风险增加。未来的研究应重点确定心血管风险的决定因素，以便进行适当的风险分层。
