MEDEIROS, Iluska Almeida Carneiro Martins de.; MEDEIROS, Paula Frassinetti Vasconcelos de.
Resumo:
The mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by deficiency in the
arylsulfatase B lysosomes enzyme , which results in glycosaminoglycan (GAG) accumulation in
various tissues, even the cardiovascular one. Aiming at describing the cardiovascular changes, a
major death cause in MPSVI, six patients (aged 4 to 13 years) were evaluated by physical
examination, electrocardiogram and echocardiogram. All patients, except younger one (aged 4)
presented heart murmur and echocardiographic changes. For all patients, electrocardiogram showed
cardiac axis deviation to the right associated with left atrial overload (1 / 6) or conduct disturbance (1
/ 6).