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Left ventricular assessment in patients with mucopolysaccharidosis using conventional echocardiography and myocardial

Mirela F A Andrade1, Isabel Cristina B Guimarães2, Angelina X Acosta3

  • 1Universidade Federal da Bahia (UFBA), Programa de Pós Graduação em Processos Interativos dos Órgãos e Sistemas (PPGPIOS), Salvador, BA, Brazil.

Jornal De Pediatria
|June 30, 2018
PubMed

Insights

Mucopolysaccharidosis patients often have cardiac issues. Early enzyme replacement therapy is linked to better heart function, as shown by echocardiography and strain analysis.

Area of Science:

  • Cardiology
  • Genetics
  • Rare Diseases

Background:

  • Mucopolysaccharidosis (MPS) involves intralysosomal glycosaminoglycan deposition, frequently causing cardiovascular impairment.
  • Cardiac manifestations in MPS are often underestimated due to multi-organ involvement.
  • Enzyme replacement therapy (ERT) is available for specific MPS types (I, II, IV, VI).

Purpose of the Study:

  • To assess left ventricular (LV) function using conventional echocardiography and LV global longitudinal strain (GLS) in MPS patients.
  • To compare LV function alterations in patients receiving ERT versus those with different treatment initiation ages.

Main Methods:

  • Descriptive, outpatient-based study.
  • Conventional echocardiography and LV global longitudinal strain measurements were performed.
  • Analysis focused on comparing patient groups based on ERT status and age at therapy start.

Main Results:

  • Sixteen MPS patients (median age 14.2 years) were evaluated.
  • Left ventricular hypertrophy (LVH) was present in 56.2% of patients.
  • While conventional systolic function was preserved, 56.2% showed altered LV GLS; LVH and late ERT initiation were associated with worse LV GLS.

Conclusions:

  • Echocardiographic abnormalities, including LV geometry changes and subclinical dysfunction, are common in MPS.
  • Delayed ERT initiation correlates with poorer LV GLS, highlighting the importance of early diagnosis and treatment.
  • Advanced echocardiographic techniques like GLS can enhance patient monitoring.
Abstract

Keywords:
Cardiac function testsEchocardiographyEcocardiografiaEnzyme replacement therapyMucopolissacaridoseMucopolysaccharidosisTerapia de reposição enzimáticaTestes de função cardíaca

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