Cardiometabolic insights in cystic fibrosis and highly effective modulator therapies: a narrative review

Alongside pulmonary and nutritional benefits, highly effective modulator therapies (HEMT) can promote an increase in body fat and raise the prevalence of overweight/obesity, dyslipidemia, and hypertension in patients with cystic fibrosis. These metabolic changes will theoretically elevate cardiovascular risk in a population that has largely been unaffected by such complications until now. A rise in the prevalence of metabolic syndrome among HEMT-treated patients is expected, which has already prompted a review of dietary recommendations. Thus, a distinction must be made between patients with several years of care experience and those, especially younger patients or those with a late diagnosis of cystic fibrosis, who have not been educated on the principle of a high-calorie diet. Whether these metabolic changes will result in a greater incidence of cardiovascular events remains uncertain, underscoring the need to validate predictive risk scores in this context.