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.