title: Cardiac amyloidosis presenting with prolonged QT interval and recurrent polymorphic ventricular tachycardia.
abstract: Cardiac amyloidosis results in severely symptomatic heart failure that has a poor prognosis because of the development of a restrictive cardiomyopathy. The diagnosis of cardiac amyloidosis is often delayed because of nonspecific signs and symptoms. We report the case of a 66-year-old woman who had been diagnosed with sick sinus syndrome and presented 5 months later with a long QT interval and recurrent polymorphic ventricular tachycardia. The diagnosis of cardiac amyloidosis was confirmed upon analysis of endomyocardial biopsy results. The patient was subsequently diagnosed with and treated for underlying plasma cell myeloma and later died of cardiac arrest. This atypical presentation of cardiac amyloidosis underscores the need to consider it in the differential diagnosis of patients who have ventricular arrhythmias. To our knowledge, the combination of long QT interval and polymorphic ventricular tachycardia has not been previously reported in association with amyloid heart disease.
authors.affiliation: Division of Cardiology, The Johns Hopkins Hospital, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA. naggarw2@jhmi.edu
authors.forename: Nisha A
authors.initials: NA
authors.lastname: Gilotra
authors.forename: Grant V
authors.initials: GV
authors.lastname: Chow
authors.forename: Oscar H
authors.initials: OH
authors.lastname: Cingolani
mesh_terms: D000200:Action Potentials; D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D001706:Biopsy; D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D004452:Echocardiography; D004562:Electrocardiography; D017809:Fatal Outcome; D005260:Female; D006323:Heart Arrest / Q000209:etiology; D006329:Heart Conduction System / Q000503:physiopathology*; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D012008:Recurrence; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D013997:Time Factors; D000098850:Polymorphic Catecholaminergic Ventricular Tachycardia
keywords: Amyloidosis/complications/diagnosis/etiology; cardiomyopathies/diagnosis/therapy; diagnosis, differential; tachycardia, ventricular/etiology/therapy