AHCM represents one end of a morphologic spectrum of HCM in which ventricular hypertrophy is principally confined to the left ventricular apex. No etiologic basis for the disease has been identified. Japanese patients with AHCM tend to be male, typically older at the age of presentation and have hypertrophy more often exclusively limited to the apical region. In Caucasian patients there is less male predominance and a wider range in age at the time of presentation. The prognosis of Caucasian patients may be less favorable than that of Japanese subjects due to more extensive hypertrophy away from the apex. The majority of patients have symptoms that are stable over time. In asymptomatic patients, the presence of deep symmetric T wave inversion suggests the possibility of AHCM. Echocardiography is essential for diagnosing apical hypertrophy and excluding more diffuse forms of myopathy with LVOT obstruction. Therapy of patients with AHCM is targeted toward relief of symptoms due to diastolic dysfunction or ischemia. The prognosis of patients with hypertrophy confined to the apex is good, whereas those with more extensive hypertrophy may have a less favorable prognosis.
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine