Case Report

Hypertrophic Cardiomyopathy and Symptomatic Conduction System Disease in Cardiac Amyloidosis

Authors: Praveen Garg, MD, Ruchi Gupta, MD, David H. Hsi, MD, Lucy A. Sheils, MD, Michael R. DiSalle, MD, Timothy J. Woodlock, MD

Abstract

Cardiac involvement is the most important prognostic factor in primary amyloidosis (AL). The clinical presentation of amyloid cardiomyopathy is varied and may manifest as heart failure, brady or tachyarrhythmias, syncope, angina and rarely with features of hypertrophic cardiomyopathy and advanced symptomatic conduction system disease. The management of amyloid cardiomyopathy has always been a dilemma, as most of the drugs used in congestive heart failure are contraindicated.


This report describes a 70-year-old woman who presented with syncope, severe diastolic heart failure, features of hypertrophic cardiomyopathy and severe symptomatic conduction system disease requiring a pacemaker. Amyloidosis was diagnosed on endomyocardial biopsy after abdominal fat aspirate was negative for amyloid. The patient eventually expired due to end-stage congestive heart failure.


Key Points


* Amyloid cardiomyopathy can present as heart failure, syncope, arrhythmias or angina.


* Hypertrophic cardiomyopathy and advanced symptomatic conduction system disease are less commonly seen in cardiac amyloidosis.


* Cardiac involvement is more common and more severe in primary amyloidosis.


* Cardiac biopsy is an important diagnostic tool in patients with symptoms suggestive of cardiac amyloidosis, especially when extracardiac tissue is negative for amyloid.

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