Summary

Apical hypertrophic cardiomyopathy (Yamaguchi syndrome) is a rare subtype of hypertrophic cardiomyopathy. The syndrome is more common in Japan where it was first described. Outside Asia, it is a very rare cause of hypertrophic cardiomyopathy. Apical hypertrophic cardiomyopathy is usually detected incidentally and has a good long-term outcome. We present a case of apical hypertrophic cardiomyopathy in an African-American patient manifesting as atrioventricular nodal re-entrant tachycardia.