Abstract
Cystic fibrosis (CF) is an autosomal recessive disease primarily affecting the respiratory system and gastrointestinal system. The life expectancy of patients with CF has significantly improved due to medical advancement and the effective use of screening techniques. However, new challenges have emerged. Particularly those involving cardiovascular pathology. This study aims to provide a better understanding of the different mechanisms that cause cardiovascular complications in patients with CF, which would help find an efficient treatment that not only prolongs survival but also improves their quality of life. This study extensively reviews different theories such as right ventricular hypertrophy due to lung pathology, ventricular interdependence, the association of nutritional deficiencies and severe cystic fibrosis transmembrane conductance regulator (CFTR) genotypes with myocardial fibrosis, effects of hypoxia, recurrent infections, and systemic inflammation of the heart and blood vessels that explain the direct or indirect involvement of the cardiovascular system in CF. For this review, 258 articles were retrieved from PubMed and Google Scholar. Out of which, a total of 12 high-quality articles were selected using appropriate quality assessment tools and preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The result of this study suggests that early detection of cardiovascular dysfunction can improve the survival rate of the patient. Furthermore, this study could aid future researchers in the exploration of various best screening modality techniques for the early detection of cardiovascular dysfunction.
Highlights
BackgroundCystic fibrosis (CF) is the most prevalent fatal inherited disease among Caucasians [1]
Another experiment done on the cardiomyocytes of neonatal mice [13,14] has revealed that CFTR is involved in regulating cardiomyocyte contraction rate
CF=Cystic fibrosis, left ventricular ejection fraction (LVEF)=Left ventricular ejection fraction, LVF=Left ventricular function. The review of these studies concludes that the cardiovascular system is directly or indirectly involved in CF
Summary
Cystic fibrosis (CF) is the most prevalent fatal inherited disease among Caucasians [1]. It is caused by defects in the CF gene located on the seventh chromosome, which encodes for the cystic fibrosis transmembrane conductance regulator (CFTR). A defect in CFTR results in reduced Cl- secretion and increased reabsorption of sodium ion (Na+) and water across the epithelium. The increased viscosity of secretions in the respiratory tract, sweat glands, gastrointestinal tract, pancreas, and other exocrine tissues, makes these secretions difficult to clear. This leads to recurrent respiratory infections, insufficient pancreatic enzymes, and accompanying complications in patients with CF. The major cause of death in CF is end-stage lung disease
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