An adult cystic fibrosis patient presenting with persistent dyspnea: case report

Abstract

BackgroundPersistent dyspnea is a common finding in the cystic fibrosis patient that typically leads to further work up of an alternative pulmonary etiology. Adult cystic fibrosis patients; however, are growing in numbers and they are living into the ages in which coronary artery disease becomes prevalent. Coronary disease should be included in the consideration of diagnostic possibilities.Case presentationA 52-year-old white male with cystic fibrosis was evaluated for exertional dyspnea associated with vague chest discomfort. Diagnostic testing revealed normal white blood cell, hemoglobin and platelet count, basic metabolic panel, fasting lipid profile, HbA1c, with chest radiograph confirming chronic cystic findings unchanged from prior radiographs and an electrocardiogram that revealed sinus rhythm with left anterior fascicular block. Stress thallium testing demonstrated a reversible anteroseptal perfusion defect with a 55% left ventricular ejection fraction. Heart catheterization found a 99% occlusion of the left anterior descending artery extending into the two diagonal branches, with 100% obstruction of the left anterior descending artery at the trifurcation and 70% lesion affecting the first posterior lateral branch of the circumflex artery.ConclusionThis case report represents the first description in the medical literature of a cystic fibrosis patient diagnosed with symptomatic coronary artery disease. Applying a standard clinical practice guide proved useful toward evaluating a differential diagnosis for a cystic fibrosis patient presenting with dyspnea and chest discomfort.

Increased augmentation index in patients with cystic fibrosis

“…However, recent reports of myocardial infarction [34,35] and hypertension [36] in non-transplanted patients challenge this; whilst our data highlights that the traditional peripheral brachial BP assessment may be normal in the presence of altered large artery haemodynamics. Although our findings need verifying in longitudinal studies to determine the long-term clinical implications of the increased AIx, it is important to identify this vascular ageing at the earliest possible stage.…”

Metabolic abnormalities in adults with cystic fibrosis

“…The prevalence of CFRD increases with age, 5 and the rate of microvascular complications of CFRD is comparable to that of type I diabetes mellitus 6 . On the other hand, macrovascular complications are uncommon in this population, although there are reports of coronary artery disease in patients with CF 7,8 …”

Prevalence of dyslipidemia in adults with cystic fibrosis

“…Microvascular disease is seen in patients with CFRD, although to a lesser degree than in the non-CF diabetic population [11]. There have been individual case reports of CF adults with ischemic heart disease, but these reports are infrequent [6].…”

“…In the non-CF population, a high fat diet is associated with the development of dyslipidemia, which is a well-recognized risk factor for atherosclerotic disease [5]. The prevalence of ischemic heart disease in the CF population is reported to be lower than the general population [6]. A high fat, high calorie diet was first introduced in the Toronto CF clinic in the early 1970s [7] therefore many patients have now lived with this diet for over 40 years.…”