Assessment of cardiac function by speckle tracking echocardiography in children with osteogenesis imperfecta.

Abstract

We aimed to assess the subclinical cardiac dysfunction in children with osteogenesis imperfecta (OI) using two-dimensional speckle tracking echocardiography (2D-STE). This cross-sectional study was carried out on 30 children with OI and 30 healthy children of matched age and sex. All children underwent conventional and tissue Doppler echocardiography, as well as 2D-STE. Height, weight, and hemoglobin were significantly lower in the patient group than in the control group. Aorta (AO), left atrial diameter (LAD), left ventricular end-systolic diameter (LVESD), left ventricular end-diastolic diameter (LVEDD), right ventricular diameter (RVD), and mean pulmonary artery (MPA) were significantly larger in the patient group compared to the control group. Mitral E/é, LV S, LV 2D-global longitudinal strain (2D-GLS), TAPSE, RV S, and RV 2D-GLS were significantly lower in the patient group than the control group. There was a significant positive correlation between the clinical severity of disease and (AO, LAD, LVESD, LVEDD, MPA, and RVD). There was a significant negative correlation between the severity of disease and LV 2D-GLS, RV é/á, and RV 2D-GLS. Children with OI have subclinical LV and RV systolic and diastolic dysfunction, and these abnormalities were correlated with the clinical severity of the disease. Osteogenesis imperfecta (OI) is an inherited disease of abnormal collagen 1 with extra-skeletal involvement, such as cardiovascular complications. Aortic dilatation and valvular dysfunction have been reported in adult patients with OI. However, right and left ventricular function assessment using two-dimensional speckle tracking echocardiography has not been studied in pediatrics before. We found that children with OI have subclinical LV and RV systolic and diastolic dysfunction, and these abnormalities are correlated with the clinical severity of the disease. Regular echocardiographic follow-up is recommended for these patients.