Cardiac involvement with amyloidosis: mechanisms of disease, diagnosis and management

Constantin B Marcu, Hans W Niessen, Aernout M Beek, Wessel P Brouwer, Lourens F Robbers, Albert C Van Rossum

Research output: Contribution to journalReview articleAcademicpeer-review

Abstract

The amyloidoses represent a group of clinical disorders of diverse etiologies that have as a common pathophysiologic denominator the deposition of misfolded protein based amyloid fibrils in the interstitial space of various organs. They are uncommon diseases with protean clinical presentations. Cardiac involvement is the determining factor for a patient's prognosis. Clinicians have to maintain a high index of suspicion and actively search for signs and symptoms of cardiac involvement in patients with preexisting conditions known to be associated with the development of amyloidosis. Early diagnosis and accurate fibril typing are the first steps in managing the disease. Judicious use of various diagnostic modalities such as serum markers and imaging studies, and good communication among all the physicians involved in the care of these sick and frail patients, are keys to a better outcome.

Original languageEnglish
Pages (from-to)581-90
Number of pages10
JournalConnecticut medicine
Volume75
Issue number10
Publication statusPublished - 6 Jan 2012

Cite this

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title = "Cardiac involvement with amyloidosis: mechanisms of disease, diagnosis and management",
abstract = "The amyloidoses represent a group of clinical disorders of diverse etiologies that have as a common pathophysiologic denominator the deposition of misfolded protein based amyloid fibrils in the interstitial space of various organs. They are uncommon diseases with protean clinical presentations. Cardiac involvement is the determining factor for a patient's prognosis. Clinicians have to maintain a high index of suspicion and actively search for signs and symptoms of cardiac involvement in patients with preexisting conditions known to be associated with the development of amyloidosis. Early diagnosis and accurate fibril typing are the first steps in managing the disease. Judicious use of various diagnostic modalities such as serum markers and imaging studies, and good communication among all the physicians involved in the care of these sick and frail patients, are keys to a better outcome.",
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Cardiac involvement with amyloidosis : mechanisms of disease, diagnosis and management. / Marcu, Constantin B; Niessen, Hans W; Beek, Aernout M; Brouwer, Wessel P; Robbers, Lourens F; Van Rossum, Albert C.

In: Connecticut medicine, Vol. 75, No. 10, 06.01.2012, p. 581-90.

Research output: Contribution to journalReview articleAcademicpeer-review

TY - JOUR

T1 - Cardiac involvement with amyloidosis

T2 - mechanisms of disease, diagnosis and management

AU - Marcu, Constantin B

AU - Niessen, Hans W

AU - Beek, Aernout M

AU - Brouwer, Wessel P

AU - Robbers, Lourens F

AU - Van Rossum, Albert C

PY - 2012/1/6

Y1 - 2012/1/6

N2 - The amyloidoses represent a group of clinical disorders of diverse etiologies that have as a common pathophysiologic denominator the deposition of misfolded protein based amyloid fibrils in the interstitial space of various organs. They are uncommon diseases with protean clinical presentations. Cardiac involvement is the determining factor for a patient's prognosis. Clinicians have to maintain a high index of suspicion and actively search for signs and symptoms of cardiac involvement in patients with preexisting conditions known to be associated with the development of amyloidosis. Early diagnosis and accurate fibril typing are the first steps in managing the disease. Judicious use of various diagnostic modalities such as serum markers and imaging studies, and good communication among all the physicians involved in the care of these sick and frail patients, are keys to a better outcome.

AB - The amyloidoses represent a group of clinical disorders of diverse etiologies that have as a common pathophysiologic denominator the deposition of misfolded protein based amyloid fibrils in the interstitial space of various organs. They are uncommon diseases with protean clinical presentations. Cardiac involvement is the determining factor for a patient's prognosis. Clinicians have to maintain a high index of suspicion and actively search for signs and symptoms of cardiac involvement in patients with preexisting conditions known to be associated with the development of amyloidosis. Early diagnosis and accurate fibril typing are the first steps in managing the disease. Judicious use of various diagnostic modalities such as serum markers and imaging studies, and good communication among all the physicians involved in the care of these sick and frail patients, are keys to a better outcome.

KW - Amyloidosis

KW - Cardiomyopathies

KW - Death, Sudden, Cardiac

KW - Diagnostic Imaging

KW - Early Diagnosis

KW - Electrocardiography

KW - Humans

KW - Microscopy, Electron

KW - Myocardium

KW - Prognosis

KW - Journal Article

KW - Review

M3 - Review article

VL - 75

SP - 581

EP - 590

JO - Connecticut medicine

JF - Connecticut medicine

SN - 0010-6178

IS - 10

ER -