Extract
The precise mechanism of sudden cardiac arrest in patients with AL-type cardiac amyloidosis is not well understood and often attributed to pulseless electrical activity, thereby implying a lack of benefit of implantable cardioverter defibrillator (ICD) in this patient population. We report on a 60-year-old male undergoing treatment for AL-type biopsy proven cardiac amyloidosis who had undergone prophylactic placement of an ICD following an episode of syncope in association with impaired left ventricular systolic function (ejection fraction of 20%), who presented 3 years later with multiple ICD firings. Device interrogation demonstrated several episodes of both monomorphic ventricular tachycardia and ventricular fibrillation, all successfully terminated by the ICD (Panels A and B). He was discharged subsequently from hospital on amiodarone and has experienced no further shocks during follow-up.
This case highlights the role of sustained ventricular arrhythmias (both monomorphic ventricular tachycardia and ventricular fibrillation) as a mechanism of sudden death in patients with cardiac amyloidosis and suggests that ICD therapy may be appropriate in selected patients.
