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 60
- Downloads 0
Magnesium sulfate infusion for acute asthma in the emergency department
Objectives To describe the role of intravenous magnesium sulfate (MgSO4) as therapy for acute severe asthma in the pediatric emergency department (ED). Source Publications were searched in the PubMed and Cochrane databa...
Predictive factors of mortality in pediatric patients with acute renal injury associated with sepsis
Objective To evaluate the prognosis factors of children with sepsis and acute kidney injury. Methods This was a retrospective study of children with sepsis and acute kidney injury that were admitted to the pediatric int...
Risk factors for central venous catheter-related infections in a neonatal population – systematic review
Objective This was a systematic review of the incidence density and risk factors for central venous catheter-related infections in a neonatal population. Data source The MEDLINE, Embase, Cochrane, BDENF, SciELO, and LIL...
Evaluation of child development: beyond the neuromotor aspect
Objective To review the epidemiology and update the scientific knowledge on the problems of development and behavior in childhood, and the recommendations for the role of the pediatrician in identifying and managing dela...
Obese fathers lead to an altered metabolism and obesity in their children in adulthood: review of experimental and human studies
Objective To discuss the recent literature on paternal obesity, focusing on the possible mechanisms of transmission of the phenotypes from the father to the children. Sources A non-systematic review in the PubMed databa...