Cardiac intramural arteriovenous malformations presented as ventricular tachycardia and fibrillation storm.

We report a case of cardiac intramural arteriovenous malformations (AVMs) misdiagnosed as hypertrophic cardiomyopathy and presented as life-threatening ventricular arrhythmia storm that was successfully controlled by cardiac sympathetic denervation. A 46-year-old male patient with an implantable cardioverter-defibrillator was admitted for recurrent ventricular tachycardia requiring repeated shock refractory to antiarrhythmic drugs. Although the patient was previously diagnosed with hypertrophic cardiomyopathy, multimodality imaging studies showed large left ventricular intramural AVMs, potentially representing arrhythmogenic substrates. Life-threatening ventricular arrhythmia storm, which could not be controlled by radiofrequency catheter ablation and therapeutic hypothermia. However, cardiac sympathetic denervation surgery successfully controlled the ventricular arrhythmia storm.