Abstract
Cardiac involvement is by far the most relevant factor impacting poor outcomes of patients with systemic light-chain (AL) amyloidosis. Median survival of patients with symptomatic cardiac AL amyloidosis is less than 6 months. Approximately two-thirds of these patients die suddenly due to ventricular arrhythmias and electromechanical dissociation. We report a 56-year-old female with very severe cardiac AL amyloidosis (NT-proBNP 13,355 ng/l, troponin T 0.16 μg/l, and systolic blood pressure 100 mmHg), who was successfully treated with diuretics and an implantable cardioverter-defibrillator (ICD) and has survived for more than 4 years, to date. During the 4-year period after receiving the ICD, she experienced several episodes of sustained ventricular tachycardia and ventricular fibrillation, all successfully terminated by anti-tachycardia pacing or electrical shock. The benefit of ICD for cardiac AL amyloidosis is unclear since there have been only a few reports of successful use of this therapy for patients with cardiac AL amyloidosis. Recently, new treatment options for AL amyloidosis, such as bortezomib and lenalidomide, have shown high response rates and improved outcomes. It is important to identify those cardiac amyloidosis patients who might be more likely to benefit from ICD implantation.
