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Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia

A 73-year-old male with long-standing Waldenstrom's macroglobulinemia complicated with systemic amyloidosis presented with a witnessed syncopal episode. He had complaints of orthostatic dizziness and palpitations for few months. Orthostatic hypotension and peripheral neuropathy were demonstrate...

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Autores principales: Jacob, Aasems, Raj, Rishi, Walkow, Warren
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5485269/
https://www.ncbi.nlm.nih.gov/pubmed/28695027
http://dx.doi.org/10.1155/2017/8795213
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author Jacob, Aasems
Raj, Rishi
Walkow, Warren
author_facet Jacob, Aasems
Raj, Rishi
Walkow, Warren
author_sort Jacob, Aasems
collection PubMed
description A 73-year-old male with long-standing Waldenstrom's macroglobulinemia complicated with systemic amyloidosis presented with a witnessed syncopal episode. He had complaints of orthostatic dizziness and palpitations for few months. Orthostatic hypotension and peripheral neuropathy were demonstrated on physical examination. EKG, 24-hour Holter monitoring, and 2D echocardiogram were unremarkable. MRI of the brain ruled out stroke. Patients with amyloidosis can develop cardiovascular disease through amyloid cardiomyopathy, small vessel disease, conduction defects, pericardial effusion, or autonomic denervation. After ruling out other life-threatening causes, Ewing's battery of tests was done to rule out cardiac autonomic neuropathy. Two heart rate tests and one blood pressure test were abnormal which indicated severe cardiac autonomic neuropathy. Cardiac autonomic neuropathy can mask symptoms of acute coronary syndrome and hence early diagnosis using the simple bedside maneuver is beneficial. The test is also important for prognostication. Absence of augmentation of cardiac output from inadequate autonomic stimulation will lead to postural hypotension, exercise intolerance, and tachycardia. There may be no change in heart rate with Valsalva or deep breathing both of which increase parasympathetic tone. As the condition progresses, it may result in cardiac denervation which can result in silent myocardial infarction, syncope, and sudden death.
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spelling pubmed-54852692017-07-10 Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia Jacob, Aasems Raj, Rishi Walkow, Warren Case Rep Hematol Case Report A 73-year-old male with long-standing Waldenstrom's macroglobulinemia complicated with systemic amyloidosis presented with a witnessed syncopal episode. He had complaints of orthostatic dizziness and palpitations for few months. Orthostatic hypotension and peripheral neuropathy were demonstrated on physical examination. EKG, 24-hour Holter monitoring, and 2D echocardiogram were unremarkable. MRI of the brain ruled out stroke. Patients with amyloidosis can develop cardiovascular disease through amyloid cardiomyopathy, small vessel disease, conduction defects, pericardial effusion, or autonomic denervation. After ruling out other life-threatening causes, Ewing's battery of tests was done to rule out cardiac autonomic neuropathy. Two heart rate tests and one blood pressure test were abnormal which indicated severe cardiac autonomic neuropathy. Cardiac autonomic neuropathy can mask symptoms of acute coronary syndrome and hence early diagnosis using the simple bedside maneuver is beneficial. The test is also important for prognostication. Absence of augmentation of cardiac output from inadequate autonomic stimulation will lead to postural hypotension, exercise intolerance, and tachycardia. There may be no change in heart rate with Valsalva or deep breathing both of which increase parasympathetic tone. As the condition progresses, it may result in cardiac denervation which can result in silent myocardial infarction, syncope, and sudden death. Hindawi 2017 2017-06-13 /pmc/articles/PMC5485269/ /pubmed/28695027 http://dx.doi.org/10.1155/2017/8795213 Text en Copyright © 2017 Aasems Jacob et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Jacob, Aasems
Raj, Rishi
Walkow, Warren
Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title_full Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title_fullStr Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title_full_unstemmed Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title_short Systemic Amyloidosis and Cardiac Autonomic Neuropathy Associated with Waldenstrom's Macroglobulinemia
title_sort systemic amyloidosis and cardiac autonomic neuropathy associated with waldenstrom's macroglobulinemia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5485269/
https://www.ncbi.nlm.nih.gov/pubmed/28695027
http://dx.doi.org/10.1155/2017/8795213
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