Assessment of right heart fonction in children with mild cystic fibrosis

Assessment of right heart function in children with mild cystic fibrosis Objective: There are no studies in pulmonary hypertension, cor pulmonale and right ventricular (RV) deficiency associated with CF in children with mild cystic fibrosis (CF). In this study, we aimed to determine echocardiographic changes in pulmonary artery pressure, right ventricular anatomy and function in mild CF children. Methods: The study group consisted of 40 pediatric patients with mild CF (FEV1>70% and Modifiye Shwachman-Kulczycki score >71) registrated at the Pediatric Pulmonolgy outpatient clinic. The control group was 40 healthy children of the same age and sex. Right ventricular anatomy in patient and control groups; M-mode and right ventricular end-diastolic diameter (RVEDD), right ventricular anterior wall thickness (RVAW), right ventricular systolic functions; tricuspit annular plane systolic excursion (TAPSE), tissue Doppler peak systolic tricuspit annular velocity (ST), right ventriculer fractional area change (RVFA), estimated pulmonary artery pressure in patients; right ventriculer pre-ejection period (RPEP), right ventriculer acceleration time (ACT), right ventriculer ejection time (RVET), and their ratio to each other RPEP/RVET, ACT/RVET were evaluated. The obtained echocardiographic data were statistically compared in the patient and control groups. Findings: In the patient parameters showed statistically significant difference in right ventricular anatomic disorder (RVAW, RVEDD, and BSA ratios) and RV systolic functions (TAPSE, ST) and diastolic functions (tricuspit E-wave, A-wave, E/A ratio) compared to the control group (p <0.001). However, no difference was found between the nomogram values for the age group (p> 0.05). x In the patient group, parameters indicating elevation of pulmonary artery pressure (RVET, ACT, RPEP) and RVFA parameters used for measuring systolic function were different from control group and for age nomogram values (p<0.001). Conclusion: Pulmonary artery pressure elevation, right ventricular failure, and cor pulmonale in children with mild CF begin early in childhood. In addition to routine echocardiographic measurements used in evaluating RV in children with mild CF, we recommend using RPEP, ACT, RVET echocardiographic parameters and RVFA, which are used to estimate pulmonary artery pressure.