Left ventricular perfusion deficit in patients with cystic fibrosis

Wolf, Daniël De; Franken, Philippe; Piepsz, Amy; Dab, I.

doi:10.1002/(sici)1099-0496(199802)25:2<93::aid-ppul4>3.0.co;2-o

Cited by 13 publications

(3 citation statements)

References 17 publications

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“…There is, however, evidence for cardiac involvement with manifestation during exercise in CF. De Wolf et al [16] studied left ventricular perfusion during exercise in 18 patients with CF. Among these, six showed defects in perfusion.…”

Section: Introductionmentioning

confidence: 72%

“…Further reports indicate fibrotic changes in the myocardium of patients with CF [17,18]. Anyhow, neither De Wolf et al [16] nor any of the other studies we could find report on arrhythmias during exercise in CF. Despite this missing evidence, we hypothesized that exercise-induced cardiac dysrhythmia does occur in patients with CF and that exercise-induced hypoxemia would be a risk factor.…”

Section: Introductionmentioning

confidence: 99%

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Exercise-induced hypoxemia and cardiac arrhythmia in cystic fibrosis

Ruf

Hebestreit

2009

Journal of Cystic Fibrosis

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Section: Introductionmentioning

confidence: 72%

Section: Introductionmentioning

confidence: 99%

Exercise-induced hypoxemia and cardiac arrhythmia in cystic fibrosis

Ruf

Hebestreit

2009

Journal of Cystic Fibrosis

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“…Many studies did not describe left ventricular dysfunction, though there is some evidence that it might be present during exercise in the pediatric CF population, which seemed to be associated with lung disease severity ( 27 ). Interestingly, there are several case reports describing coronary artery disease in pwCF and possible atherosclerosis ( 28 – 30 ).…”

Section: Cardiovascular Diseasementioning

confidence: 99%

What the future holds: cystic fibrosis and aging

Blankenship,

Landis,

Harrison Williams

et al. 2024

Front. Med.

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Cystic fibrosis (CF) is one of the most common genetic diseases with around 70,000 affected patients worldwide. CF is a multisystem disease caused by a mutation in the CF transmembrane conductance regulator gene, which has led to a significant decrease in life expectancy and a marked impairment in the quality of life for people with CF (pwCF). In recent years, the use of highly effective CFTR modulator therapy (HEMT) has led to improved pulmonary function, fewer CF exacerbations, lower symptom burden, and increased weight. This has coincided with an increased life expectancy for pwCF, with mean age of survival being now in the 50s. This being a major breakthrough, which the CF population has hoped for, pwCF are now facing new challenges by growing old with a chronic respiratory disease. In this mini review, we are attempting to summarize the current knowledge of the aging process and its effect on CF disease and its manifestations including new developments, the current research gaps and potential future developments in the field to allow healthy aging for the CF community.

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