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.


METHODS
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.


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


RESULTS
Pharmacological antiarrhythmic therapy and cardioverter-defibrillator implantation. Chemotherapy was planned but, despite intervention, the patient died before this treatment could begin.
Wright
Ben L C
BL
Papworth Hospital, Papworth Everard. dr_b_wright@yahoo.co.uk
Grace
Andrew A
AA
Goodman
Hugh J B
HJ
D000686:Amyloidosis / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D006333:Heart Failure; D006801:Humans; D008875:Middle Aged