Heart involvement in children and adults with cystic fibrosis: correlation with pulmonary indexes and inflammation markers

Background: Cardiovascular involvement in Cystic Fibrosis (CF) is a not rare condition, although the prevalence of subclinical pulmonary hypertension (PH) and cardiac dysfunction is not known in the early stages of CF progression. The aim of our study was to assess cardiac involvement in children and adults affected by cystic fibrosis compared with healthy subjects of same age using echocardiography. Methods: Fifty-five patients, 25 adults and 30 children completed the study. We assessed FEV1 (Forced Expiratory Volume in one second), and carried out colour Doppler-echocardiography evaluating ejection fraction (EF) measurement of left ventricle, tricuspid annular plane systolic excursion (TAPSE) of right ventricle and pulmonary artery pressure (PAP). We compared the auxological, respiratory and cardiologic data with those of 16 adults and 34 children of the same age. Results: We discovered significantly different values of PAP between patients and controls in both children (p = 0.0001, r = - 0.62) and adults (p = 0.0001, r = - 0.63), whereas the EF and TAPSE showed significantly different values in only adults (p. = 0.0023 and p. = 0.0194 respectively).We found in both children and adults with CF an inverse correlation between PAP and FEV1 (p = 0.000, p = 0.001), Erythrocyte Sedimentation Rate (ESR) and FEV 1 (p = 0.015, r = - 0.43; p = 0.009, r = - 0.51), and highly sensitive C-reactive protein (hs-CRP) and FEV 1 (p = 0.007, r = - 0.48; p = 0.001, r = - 0.60). In adults we also detected direct correlation between PAP and hs-CRP (p = 0.008, r = 0.51) and PAP and ESR (p = 0.009, r = 0.51). Conclusions: In paediatric-aged CF patients there are already early signs of potential heart impairment, represented by an increase of pulmonary blood pressure, and in adult age the systolic function of right ventricle may be impaired. We hypothesise that such cardiac impairments may gradually arise due to preceding chronic inflammation related to prior degeneration of lung function and thus it is very important to keep patients clinically stable and address chronic inflammation as early as possible in the progression of CF