Yamaguchi cardiomyopathy a.k.a. apical hypertrophic cardiomyopathy (AHCM) was first described 1976 in Japanese patients.
AHCM is a variant of hypertrophic cardiomyopathy that is nonobstructive with predominant involvement of the apex of the heart.
AHCM is frequently misdiagnosed as ACS or STEMI since the typical ECG abnormalities include giant inverted T waves or ST elevation in the mid precordial leads, however coronaries are characteristically clean on cardiac catheterization.
Echocardiography classically used to diagnosis HCM may frequently miss AHCM because hypertrophy is only localized to the apex.
Nuclear magnetic resonance imaging or angiography reveals the pathognomonic "ace of spades" configuration of the left ventricle with systolic obliteration of the apical region.
Unlike HCM sudden cardiac death is very uncommon.
References
Olearczyk B, Gollol-Raju N, Menzies D. Apical Hypertrophic Cardiomyopathy Mimicking Acute Coronary Syndrome: A Case Report and Review of the Literature. Angiology Vol 59; No. 5. Oct/Nov 2008 629-631.
