Key result
Worse LV GLS associates with increased MACE risk in pediatric hypertrophic cardiomyopathy.
Why the study?
Hypertrophic cardiomyopathy is very rare in patients with Down's syndrome, with only five cases previously reported.
Observational (n=31)
No
Effect estimate: AUC 0.861 (95% CI 0.675-1.0)
p-value: p=0.003
In persons with Down's syndrome (DS), congenital cardiac defects are frequent, including atrioventricular canal defect, ventricular septal defects and atrial septal defects. However, in patients with this syndrome, hypertrophic cardiomyopathy is very rare because only five cases have been previously reported in the literature. We have identified one male 52-year-old with DS and non-obstructive cardiomyopathy. The echocardiogram of this patient showed a prevalent apical involvement of the left ventricular hypertrophy as previously reported in two adult patients with DS and hypertrophic cardiomyopathy. Although rare, hypertrophic cardiomyopathy can occur in individuals with DS and, in adult patients, the apical form is frequent.
No takes yet. Share an insight, caveat, or question.
Assenza et al. (2007) conducted an observational in Hypertrophic cardiomyopathy (n=31). Impaired longitudinal left ventricular systolic function (LV GLS and MADI) vs. Preserved or less impaired longitudinal left ventricular systolic function was evaluated on Discriminative performance of LV GLS for Major adverse cardiovascular events (MACE) (AUC 0.861, 95% CI 0.675-1.0, p=0.003). In children and adolescents with hypertrophic cardiomyopathy, worse left ventricular global longitudinal strain (LV GLS ≥ -12.1%, AUC 0.861) and lower mitral annular displacement index were significantly associated with an increased risk of major adverse cardiovascular events.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: