Coronary artery disease in patients with cystic fibrosis – A case series and review of the literature

Progressive obstructive pulmonary disease is the primary life-shortening complication in people with Cystic Fibrosis (CF); improvement in life expectancy has led to increased prevalence of non-pulmonary complications. Patients with CF are considered to be at low risk for coronary artery disease (CAD). We report here a case series of six patients with CF with and without known cystic fibrosis related diabetes (CFRD) who had acute myocardial infarction (AMI) requiring coronary stent placement. This was a heterogeneous group of patients, without a clear pattern of consistent risk factors. Interestingly, most patients in this cohort had low LDL. In this review, we discuss risk factors of cardiovascular disease (CVD) that may apply to the CF population. While CAD is rare in people with CF, it does occur. We postulate that the risk will grow with increased longevity and the increased prevalence of co-morbidities such as obesity and dyslipidemia.