Apical hypertrophic cardiomyopathy

Yamaguchi syndrome

Abstract

Introduction: apical hypertrophic cardiomyopathy (HCM) accounts for 15% of patients with HCM in Japan. However, in our country it is a rare diagnosis. In this entity, the apex is almost exclusively affected, most commonly in the left ventricle. It is diagnosed by finding apical thickening greater than 15 mm or a ratio of left ventricular apical to basal wall thickness ?1.3-1.5. It is treated symptomatically with beta blockers and automatic defibrillator implantation for primary prevention of sudden death.

Case presentation: a 59-year-old patient in whom apical HCM, or Yamaguchi syndrome, was found incidentally.

Conclusion: apical HCM is a rare entity, in many cases asymptomatic. Its prompt diagnosis allows early treatment and prevents cardiovascular outcomes, including fatal ones.