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Published on: July 16, 2020
Young patients with cystic fibrosis demonstrate subtle alterations of the cardiovascular system
Jacobien B Eising1, Cornelis K van der Ent1, Arco J Teske2
1Department of Paediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands.
Insights
Children with cystic fibrosis exhibit increased arterial stiffness and early signs of reduced heart function, even at a young age. This highlights the importance of monitoring cardiac health in pediatric cystic fibrosis patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Complications
- Cystic Fibrosis Research
Background:
- Increasing life expectancy in cystic fibrosis (CF) necessitates focus on non-pulmonary comorbidities.
- Myocardial dysfunction is observed in adult CF patients, but its early development is poorly understood.
- Cardiac function in pediatric CF patients remains under-investigated.
Purpose of the Study:
- To prospectively assess cardiac function in children with cystic fibrosis.
- To compare cardiac function between pediatric CF patients and healthy children.
- To investigate early signs of myocardial dysfunction in young CF patients.
Main Methods:
- Recruited 33 children (aged 3-12) with CF and 33 age-matched healthy controls.
- Performed lung function tests, arterial stiffness measurements (pulse wave velocity), and echocardiography.
- Utilized conventional echocardiography and myocardial deformation imaging (strain and strain rate).
Main Results:
- No significant differences in anthropometrics, lung function, or blood pressure.
- CF children showed higher arterial stiffness (p=0.049).
- Reduced right ventricular systolic function and lower global ventricular strain in CF children (p<0.01 for RV, p=0.022 for LV).
Conclusions:
- Pediatric CF patients demonstrate increased arterial stiffness from a young age.
- Early signs of diminished right and left ventricular function are present in children with CF.
- Findings underscore the need for early cardiac monitoring in pediatric CF.
Background:
As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children.
Methods:
33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study. Measurements of lung function, arterial stiffness, and echocardiography (conventional measures and myocardial deformation imaging) were performed.
Results:
There were no differences in anthropometrics, lung function and blood pressure between the two groups. The cystic fibrosis children had a higher arterial stiffness compared to the healthy children (pulse wave velocity respectively 5.76±0.57m/s versus 5.43±0.61m/s, p-value 0.049). Using conventional echocardiographic parameters for right ventricular function, Tricuspid Annular Plane Systolic Excursion) and Tissue Doppler Imaging, cystic fibrosis children had a reduced right ventricular systolic function when compared to the healthy children. After adjustment for lung function, global strains of both right and left ventricles were significantly lower in the cystic fibrosis group than in healthy children (linear regression coefficient 1.45% left ventricle, p-value 0.022 and 4.42% right ventricle, p-value <0.01). Systolic strain rate of basal segment of the left ventricle, the mid segment of the right ventricle and the apical septum were significantly lower in the cystic fibrosis children than in healthy controls.
Conclusion:
Our study suggests that already at a very young age, children with cystic fibrosis show an increased arterial stiffness and some signs of diminished both right and left ventricular function.
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