Implantation of a cardioverter-defibrillator in a patient with cardiac amyloidosis.

Background: A 49-year-old woman presented with chest pain of 10 days' duration. Initial physical examinations and laboratory investigations were normal. The patient received symptomatic treatment with beta-blockers, which continued following normal findings on coronary angiogram. About 7 months later the patient developed ventricular arrhythmias, with clinical evidence of left ventricular heart failure. Her arrhythmia symptoms persisted despite pharmacological therapy with atenolol, carvedilol and amiodarone.

Investigations: Physical examination, electrocardiography, laboratory testing, serologic testing, exercise-tolerance testing, coronary angiography, chest radiography, cardiac MRI, tongue biopsy, bone-marrow biopsy, CT scan, iodine-123-labeled serum-amyloid-P-component scintigraphy.

Diagnosis: Systemic primary amyloidosis (AL amyloidosis), with predominant cardiac involvement.

Management: Pharmacological antiarrhythmic therapy and cardioverter-defibrillator implantation. Chemotherapy was planned but, despite intervention, the patient died before this treatment could begin.