title: Life-saving implantable cardioverter defibrillator therapy in cardiac AL amyloidosis.
abstract: Cardiac involvement is the main determinant of prognosis in systemic monoclonal immunoglobulin light chain (AL) amyloidosis. Ventricular arrhythmias and sudden cardiac death are not uncommon. The electrical events that precede sudden death, and their potential to be treated effectively, remain undefined. There are no European guidelines for the use of implantable cardioverter defibrillator (ICD) in amyloidosis. ICDs in general are not usually offered to patients with a life expectancy of less than 1 year. We describe a patient who presented with cardiac AL amyloidosis who underwent prophylactic ICD implantation for the prevention of sudden cardiac death during treatment with chemotherapy, in whom life-threatening ventricular arrhythmia was successfully terminated over a 3-year period.
authors.affiliation: National Amyloidosis Centre, London, UK.
authors.forename: Ketna S
authors.initials: KS
authors.lastname: Patel
authors.affiliation: National Amyloidosis Centre, London, UK.
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: National Amyloidosis Centre, London, UK.
authors.forename: Carol J
authors.initials: CJ
authors.lastname: Whelan
authors.affiliation: National Amyloidosis Centre, London, UK.
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
mesh_terms: D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000517:prevention & control; D053840:Brugada Syndrome; D000075224:Cardiac Conduction System Disease; D009202:Cardiomyopathies / Q000209:etiology / Q000517:prevention & control*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006329:Heart Conduction System / Q000002:abnormalities; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D000075363:Immunoglobulin Light-chain Amyloidosis; D008875:Middle Aged; D011379:Prognosis; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000517:prevention & control*
chemical_list: D000682:Amyloid; D007147:Immunoglobulin Light Chains