We report cases of serious arrhythmias associated with apical hypertrophic
cardiomyopathy (AHCM), Thirty-one patients were referred to our institute t
o undergo further assessment of their AHCM from 1988 to 1999, Three patient
s with nonsustained ventricular tachycardia demonstrated an I-123-MIBG regi
onal reduction in the tracer uptake. In two patients with ventricular fibri
llation (VF), the findings from I-123-MIBG imaging revealed regional sympat
hetic denervation in the inferior and lateral regions. Electrophysiologic s
tudy demonstrated reproducible induction of VF in aborted sudden death and
presyncopal patients, resulting in the need for an implantable defibriliato
r device and amiodarone in each patient. Patients with refractory atrial fi
brillation with a rapid ventricular response suffered from serious congesti
ve heart failure. A prudent assessment and strategy in patients with this d
isease would be indispensable in avoiding a disastrous outcome.