50 male hypertension, former smoker who presents for evaluation of chest pain found to have concern for apical hypertrophic cardiomyopathy on exercise stress echocardiogram

 

ECG, : Sinus bradycardia, PACs, LVH with repolarization, HR 58, PR 184ms, QRS 96ms, QTc 449ms

 

Stress testing, Treadmill exercise stress echocardiogram with ischemic ECG changes but no
evidence of inducible myocardial ischemia by symptoms, or echo findings at a
non-diagnostic level of peak stress (achieved 81% of maximal age-predicted

heart rate) and low workload (<6.1 METS). Exercise capacity is below average
when referenced to age and gender. Normal heart rate and blood pressure
response with exercise.

Resting echocardiography demonstrates increased apical wall thickness with
spade shaped ventricle. This along with his resting ECG, are suggestive of
apical hypertrophic cardiomyopathy.

• Apical hypertrophic cardiomyopathy (AHCM) is a
  • rare form of hypertrophic cardiomyopathy (HCM)
  • thought to be confined to the Japanese population
  • also found in other populations.
    • Japan the prevalence of AHCM was 15%, of CM
    • USA the prevalence was only 3%[2].
    • AHCM may mimic other conditions,
      • apical cardiac tumors[10],
      • LV apical thrombus[
      • isolated ventricular non-compaction
      • endomyocardial fibrosis (EMF)[