Cystic fibrosis is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to dysfunction of the CFTR protein. CFTR dysfunction leads to disease in the respiratory and gastrointestinal systems. Disorders of the cardiovascular system in individuals with CF are usually attributed to secondary effects from progressive lung disease. However, CFTR has been localized to vascular endothelium and smooth muscle, suggesting that CFTR dysfunction may directly impact cardiovascular function. As treatments for CF improve and life-expectancy increases, the risk of vascular disease may increase in prevalence related to primary and secondary CFTR dysfunction, chronic systemic inflammation, nutritional health and hyperglycemia in individuals with CF related diabetes. Here we review the available literature on CF and the cardiovascular system, examining the secondary effects and evidence for direct CFTR dysfunction in the heart, aorta, pulmonary vessels, and vasculature, as well as future directions and treatment options.

囊性纤维化是一种由囊性纤维化跨膜电导调节因子（ CFTR）突变引起的遗传性疾病。) 基因，导致 CFTR 蛋白功能障碍。CFTR 功能障碍导致呼吸和胃肠系统疾病。CF 患者的心血管系统疾病通常归因于进行性肺病的继发性影响。然而，CFTR 已定位于血管内皮和平滑肌，这表明 CFTR 功能障碍可能直接影响心血管功能。随着 CF 治疗的改善和预期寿命的增加，与 CF 相关糖尿病患者的原发性和继发性 CFTR 功能障碍、慢性全身炎症、营养健康和高血糖相关的患病率可能会增加。在这里，我们回顾了有关 CF 和心血管系统的现有文献，