Key result
Patients with Mucopolysaccharidosis Type IVA exhibited significantly reduced stroke volumes (z-score -2.3) and smaller left ventricular dimensions, compensated by a 21% increase in resting heart rate.
Observational (n=54)
No
Effect estimate: z-score -2.3 (95% CI -2.63 to -1.98)
Patients with MPS IVA exhibit age-progressive disproportion of intra-thoracic organs, aortic root extension, thickened left ventricles, reduced stroke volumes, impaired diastolic filling, and compensatory increased heart rates.
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These changes may warrant cardiac surveillance in MPS IVA; leaves open prognostic implications and intervention needs.
Kampmann et al. (2016) conducted an observational in Mucopolysaccharidosis Type IVA (Morquio-A Syndrome) (n=54). Mucopolysaccharidosis Type IVA vs. Healthy infants and children was evaluated on Stroke volume index (z-score) (z-score -2.3, 95% CI -2.63 to -1.98). Patients with Mucopolysaccharidosis Type IVA exhibited significantly reduced stroke volumes (z-score -2.3) and smaller left ventricular dimensions, compensated by a 21% increase in resting heart rate.
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