Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?

A 59-year-old male presented with syncope and nonsustained ventricular tachycardia and was diagnosed to have hypertrophic obstructive cardiomyopathy. He underwent dual-chamber implantable converter defibrillator (ICD) insertion. Following ICD insertion, in view of some red flags, routine cardiac mag...

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Main Authors: Madhu Shukla, Jagdish Chander Mohan
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2024-07-01
Series:Journal of the Indian Academy of Echocardiography & Cardiovascular Imaging
Subjects:
Online Access:https://journals.lww.com/10.4103/jiae.jiae_3_24
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author Madhu Shukla
Jagdish Chander Mohan
author_facet Madhu Shukla
Jagdish Chander Mohan
author_sort Madhu Shukla
collection DOAJ
description A 59-year-old male presented with syncope and nonsustained ventricular tachycardia and was diagnosed to have hypertrophic obstructive cardiomyopathy. He underwent dual-chamber implantable converter defibrillator (ICD) insertion. Following ICD insertion, in view of some red flags, routine cardiac magnetic resonance imaging was performed, which showed biventricular hypertrophy, diffuse subendocardial gadolinium enhancement of both the ventricles, prolonged T1 and T2, and left ventricular extracellular volume fraction of 50%. A pyrophosphate scan of the heart was strongly positive both at 1 and 3 h intervals. M-band was observed on plasma protein electrophoresis. Serum and urinary immunofixation studies suggested lambda light-chain gammopathy. Multiparametric flow cytometry of the bone marrow aspirate revealed 20% plasma cells immunophenotyped to produce only light-chain immunoglobulins. The patient is on specific chemotherapy for the past 7 months and is asymptomatic.
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institution Kabale University
issn 2543-1463
2543-1471
language English
publishDate 2024-07-01
publisher Wolters Kluwer Medknow Publications
record_format Article
series Journal of the Indian Academy of Echocardiography & Cardiovascular Imaging
spelling doaj-art-76bfdff74fbc47b698a93e1e7c0dc1ee2025-01-12T10:19:14ZengWolters Kluwer Medknow PublicationsJournal of the Indian Academy of Echocardiography & Cardiovascular Imaging2543-14632543-14712024-07-018317718010.4103/jiae.jiae_3_24Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?Madhu ShuklaJagdish Chander MohanA 59-year-old male presented with syncope and nonsustained ventricular tachycardia and was diagnosed to have hypertrophic obstructive cardiomyopathy. He underwent dual-chamber implantable converter defibrillator (ICD) insertion. Following ICD insertion, in view of some red flags, routine cardiac magnetic resonance imaging was performed, which showed biventricular hypertrophy, diffuse subendocardial gadolinium enhancement of both the ventricles, prolonged T1 and T2, and left ventricular extracellular volume fraction of 50%. A pyrophosphate scan of the heart was strongly positive both at 1 and 3 h intervals. M-band was observed on plasma protein electrophoresis. Serum and urinary immunofixation studies suggested lambda light-chain gammopathy. Multiparametric flow cytometry of the bone marrow aspirate revealed 20% plasma cells immunophenotyped to produce only light-chain immunoglobulins. The patient is on specific chemotherapy for the past 7 months and is asymptomatic.https://journals.lww.com/10.4103/jiae.jiae_3_24cardiac amyloidosishypertrophic cardiomyopathysyncope
spellingShingle Madhu Shukla
Jagdish Chander Mohan
Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
Journal of the Indian Academy of Echocardiography & Cardiovascular Imaging
cardiac amyloidosis
hypertrophic cardiomyopathy
syncope
title Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
title_full Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
title_fullStr Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
title_full_unstemmed Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
title_short Cardiac Amyloidosis Masquerading as Hypertrophic Obstructive Cardiomyopathy: Co-existence or Coincidence?
title_sort cardiac amyloidosis masquerading as hypertrophic obstructive cardiomyopathy co existence or coincidence
topic cardiac amyloidosis
hypertrophic cardiomyopathy
syncope
url https://journals.lww.com/10.4103/jiae.jiae_3_24
work_keys_str_mv AT madhushukla cardiacamyloidosismasqueradingashypertrophicobstructivecardiomyopathycoexistenceorcoincidence
AT jagdishchandermohan cardiacamyloidosismasqueradingashypertrophicobstructivecardiomyopathycoexistenceorcoincidence