P1000Right ventricular function is decreased in children with cystic fibrosis.


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Abstract

Purpose: Chronic pulmonary problems in cystic fibrosis (CF) can be complicated by development of pulmonary hypertension during adulthood. In most adult CF patients both systolic and diastolic right ventricular (RV) function are compromised. Since early symptoms are often masked by pulmonary problems, cardiac symptoms only reveal themselves when RV failure develops. So far cardiac function has only been evaluated in adults and it is unknown when decrease of RV function initiates.Methods: A pilot study was performed at a tertiary referral centre for pediatric CF. The first 10 CF patients attending the outpatient clinic, aged 3-9 years, were asked to participate. Healthy controls, in the same age-range, were selected from the ongoing ‘WHISTLER’ birth cohort. RV function was evaluated using echocardiography, including blood Doppler, tissue Doppler and gray scale images from which basic diastolic and systolic RV parameters were measured. Results were compared using the independent student T-test.Results: Ten CF patients and 9 healthy controls were included in the analysis; mean age in both groups was respectively 71 and 60 months (p=0.334). The results are outlined in table 1, in all children the measured parameters were within the normal limits for their age. Conclusions: Pediatric CF patients showed a trend toward decreased systolic and -to lesser extent- diastolic RV function compared to normal children. These results may indicate that changes in the RV function occur early in life and may not exclusively present in patients with severe pulmonary problems. This raises the question whether evaluation of RV function should be routinely done earlier in life and this could have implications for treatment.

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