Left ventricular assessment in patients with mucopolysaccharidosis using conventional echocardiography and myocardial deformation by two-dimensional speckle-tracking method
Journal Title: Jornal de Pediatria - Year 2019, Vol 95, Issue 4
Abstract
Objective Mucopolysaccharidosis is a rare genetic disease characterized by the intralysosomal deposition of glycosaminoglycans. Cardiovascular impairment is a common feature. Cardiac signs and symptoms are underestimated due to the disease involvement in other organs. Enzyme replacement therapy can be used in mucopolysaccharidosis I, II, IV, and VI. Thus, the knowledge about the use of new echocardiography tools is relevant to improve the care of this population. This study aimed to describe left ventricular function assessment by conventional echocardiography and left ventricular global longitudinal strain analysis and compare the alterations in patients receiving enzyme replacement therapy and who had different ages at the start of therapy. Method Outpatient-based descriptive study. The patients were submitted to conventional echocardiography and left ventricular global longitudinal strain measurement. Results Sixteen patients were evaluated; median age of 14.2 years (SD=5.2 years). Left ventricular hypertrophy was found in nine patients (56.2%). All patients had preserved left ventricular systolic function (Simpson and Teichholz). Nine (56.2%) patients showed alterations in left ventricular global longitudinal strain. The study showed a positive association between left ventricular hypertrophy and alteration in the left ventricular global longitudinal strain, and late start of enzyme replacement therapy and alteration in the left ventricular global longitudinal strain. Conclusion Echocardiographic alterations in patients with mucopolysaccharidosis were frequently observed, especially alterations in the left ventricular geometry and subclinical dysfunction. Patients who had a late enzyme replacement therapy start showed an association with worse left ventricular global longitudinal strain values, reinforcing the need for early diagnosis and treatment. The use of new echocardiographic tools may improve the follow-up of these patients.
Authors and Affiliations
Mirela Andrade
Keywords
Related Articles
- EP ID EP612735
- DOI 10.1016/j.jped.2018.05.006
- Views 77
- Downloads 0
Screen time impairs the relationship between physical fitness and academic attainment in children
Objective The purpose of this study was twofold: to analyze the association between physical fitness and academic attainment, and to determine the influence of screen time on the association between physical fitness and...
Role of echocardiography in reducing shock reversal time in pediatric septic shock: a randomized controlled trial
Objective To evaluate the role of echocardiography in reducing shock reversal time in pediatric septic shock. Methods A prospective study conducted in the pediatric intensive care unit of a tertiary care teaching hospit...
Evaluation of respiratory dynamics by volumetric capnography during submaximal exercise protocol of six minutes on treadmill in cystic fibrosis patients
Objectives Volumetric capnography provides the standard CO2 elimination by the volume expired per respiratory cycle and is a measure to assess pulmonary involvement. Thus, the objective of this study was to evaluate the...
Prevalence and factors associated with smoking among adolescents
Objective Despite anti-smoking prevention programs, many adolescents start smoking at school age. The main objectives of this study were to determine the prevalence and risk factors associated with smoking in adolescents...
Growth of preterm low birth weight infants until 24 months corrected age: effect of maternal hypertension
Objective To evaluate the growth pattern of low birth weight preterm infants born to hypertensive mothers, the occurrence of growth disorders, and risk factors for inadequate growth at 24 months of corrected age (CA). M...