A Case Report of Cardiac Amyloidosis Initially Managed as Dilated Cardiomyopathy: Missing the obvious!

CASE STUDY, June 2016, VOL IV ISSUE I, ISSN 2042-4884
10.5083/ejcm.20424884.148 , Cite or Link Using DOI
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Dr Yerramareddy Vijaya Chandra, Dr Vellore Satyanarayanan Yogeeswari

Introduction

Amyloidosis is a rare disorder with uncertain incidence; however, in UK and US population, AL amyloidosis, the most frequently diagnosed type, has an annual incidence of 6 to 10 cases per million. The deposition of amyloid, the extracellular proteinaceous material, in the tissues results in a group of disorders called amyloidoses. The most commonly deposited amyloid material in various organ systems including heart are light chains, transthyretin and serum amyloid A.

One of the challenges in diagnosing amyloidosis early is that it commonly manifests with nonspecific symptoms of fatigue and weight loss. The diagnosis is generally considered only when symptoms are traceable to a specific organ. Cardiac amyloidosis presents initially with mild LV diastolic dysfunction, progressing to classical restrictive cardiomyopathy and finally even dilated cardiomyopathy like stage with end-stage heart failure. The disease can be mistaken in the early stages with hypertrophic cardiomyopathy and hypertensive heart disease and in the late stages with the common-garden variety of dilated cardiomyopathy. Here, we describe a case of cardiac amyloidosis, initially diagnosed and managed as dilated cardiomyopathy with inadequate response to management.