title: Isolated cardiac involvement in primary amyloidosis: presenting as sick sinus syndrome and heart failure.
abstract: Cardiac amyloidosis is an infiltrative cardiomyopathy with a grave prognosis. Its clinical manifestations include restrictive cardiomyopathy, diastolic heart failure, conduction defects, and arrhythmias. Isolated cardiac involvement and significant conduction disturbances are reported very infrequently. We report a rare case of isolated cardiac involvement in primary amyloidosis, in a 76-year-old man who initially presented with sick sinus syndrome that necessitated permanent pacemaker insertion. Subsequent symptoms of heart failure led to additional evaluation, including an endomyocardial biopsy that revealed primary cardiac amyloidosis. Medical therapy improved the patient's symptoms, and he was discharged from the hospital in stable condition. In addition to discussing the patient's case, we review the relevant medical literature.
authors.affiliation: Department of Internal Medicine (Dr. Pattanshetty), Cleveland Clinic, Cleveland, Ohio 44195; and The Heart and Vascular Center (Drs. Bhat and Lyons) and Department of Pathology (Dr. Chamberlain), MetroHealth Campus of Case Western Reserve University, Cleveland, Ohio 44109.
authors.forename: Deepak J
authors.initials: DJ
authors.lastname: Pattanshetty
authors.affiliation: Department of Internal Medicine (Dr. Pattanshetty), Cleveland Clinic, Cleveland, Ohio 44195; and The Heart and Vascular Center (Drs. Bhat and Lyons) and Department of Pathology (Dr. Chamberlain), MetroHealth Campus of Case Western Reserve University, Cleveland, Ohio 44109.
authors.forename: Pradeep K
authors.initials: PK
authors.lastname: Bhat
authors.affiliation: Department of Internal Medicine (Dr. Pattanshetty), Cleveland Clinic, Cleveland, Ohio 44195; and The Heart and Vascular Center (Drs. Bhat and Lyons) and Department of Pathology (Dr. Chamberlain), MetroHealth Campus of Case Western Reserve University, Cleveland, Ohio 44109.
authors.forename: Wendy A
authors.initials: WA
authors.lastname: Chamberlain
authors.affiliation: Department of Internal Medicine (Dr. Pattanshetty), Cleveland Clinic, Cleveland, Ohio 44195; and The Heart and Vascular Center (Drs. Bhat and Lyons) and Department of Pathology (Dr. Chamberlain), MetroHealth Campus of Case Western Reserve University, Cleveland, Ohio 44109.
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Lyons
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D002304:Cardiac Pacing, Artificial; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D004452:Echocardiography; D004562:Electrocardiography; D005500:Follow-Up Studies; D054144:Heart Failure, Diastolic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D008297:Male; D009206:Myocardium / Q000473:pathology; D012804:Sick Sinus Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy
keywords: Amyloidosis/complications/drug therapy/pathology; cardiomyopathies/complications/pathology; heart failure/diagnosis/etiology; prognosis; treatment outcome