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What Is Yamaguchi Syndrome?
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substack.com
Yamaguchi Syndrome AKA apical non-obstructive hypertrophic cardiomyopathy is thought to be an autosomal dominant condition where the LV hypertrophies and produces an “Ace of Spades” ♠️ view on angiography. The most common gene mutations include MYBPC3 and MYH7. This rare cardiomyopathy is typically seen in patients of Japanese descent but can also occur in other populations. LV Ventriculography findings 1 Ace of Spades ♠️ appearance at the end of diastole 2 Coronary angio show no occlusion ECHO
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FacebookCardioVisual
Apical H. Cardiomyopathy (ApHCM — a special variant of HCM, often called Yamaguchi syndrome. --- 1--Definition & Epidemiology A variant of hypertrophic cardiomyopathy characterized by predominant hypertrophy of the left ventricular (LV) apex, producing a "spade-like" configuration of the LV cavity on ventriculography/echo/MRI. More common in East Asian populations (esp. Japan), but also seen worldwide. Often diagnosed later in life compared to classic septal HCM. --- 2--Pathophysiology Genetic b
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