Men with Danon disease show rapid regression of massive left ventricular hypertrophy followed by dilation and hypokinesis leading to heart failure within 2 years.
This case report visually documents the rapid phenotypic transition from massive left ventricular hypertrophy to dilation and hypokinesis over just 2 years in a male patient with Danon disease.
Absolute Event Rate: 0% vs 0%
Danon disease is a rare X-linked multi-system lysosomal storage disorder. It may manifest as severe cardiomyopathy in affected individuals, with different clinical courses observed for men and women. Women may experience a hypertrophic or a dilated/hypokinetic phenotype irrespective of their disease stage. Men typically present earlier with progressive hypertrophy, and rapid regression of their hypertrophy with dilation and hypokinesis with onset of heart failure outcomes. In this case we demonstrate echocardiographic images of a young male taken just 2 years apart.
Mannion et al. (Tue,) reported a other. Men with Danon disease show rapid regression of massive left ventricular hypertrophy followed by dilation and hypokinesis leading to heart failure within 2 years.
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