Key result
A 17-year-old female with Danon disease and normal systolic function developed a left ventricular apical thrombus leading to transient ischemic attack and ischemic stroke.
Case Report (n=1)
Highlights the risk of LV apical thrombus and subsequent cerebral ischemic events in Danon disease patients even when LV systolic function is normal.
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LV apical thrombus risk warrants consideration in Danon disease despite normal systolic function; this case leaves open need for prospective screening studies.
Danon disease is a rare X-linked dominant skeletal and cardiac muscle disorder presenting with hypertrophic cardiomyopathy, Wolf-Parkinson-White syndrome, skeletal myopathy, and mild intellectual disability. Early morbidity and mortality due to heart failure or sudden death are known in Danon disease, more in males than in females. Here, we present a 17-year-old female adolescent with Danon disease and severe concentric hypertrophy with normal left ventricular (LV) systolic function, who has been complaining of intermittent headache and weakness for about 3 years, initially diagnosed with hemiplegic migraine. Subsequently, her neurological manifestation progressed to transient ischemic attack (TIA) and eventually to ischemic stroke confirmed by CT scan with 1-day history of expressive aphasia followed by persistent left side weakness and numbness. Detailed echocardiogram for the first time revealed a small LV apical thrombus with unchanged severe biventricular hypertrophy and normal systolic function. This unexpected LV apical thrombus may be associated with a wide spectrum of neurological deficits ranging from TIA to ischemic stroke in Danon disease. Possibility of cerebral ischemic events should be suspected in Danon disease when presenting with neurological deficits even with normal systolic function. Careful assessment for LV apical thrombus is warranted in such cases.
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Tsuda et al. (2017) conducted a case report in Danon disease (n=1). Left ventricular apical thrombus in Danon disease was evaluated on Transient ischemic attack and ischemic stroke. A 17-year-old female with Danon disease and normal systolic function developed a left ventricular apical thrombus leading to transient ischemic attack and ischemic stroke.
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