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Erschienen in: Netherlands Heart Journal 9/2016

Open Access 18.08.2016 | Heart Beat

An unusual case of systemic amyloid causing constrictive heart failure

verfasst von: F. A. A. Mohamed Hoesein, M. J. Swaans, L. S. Jiwa, C. A. Seldenrijk, H. W. van Es

Erschienen in: Netherlands Heart Journal | Ausgabe 9/2016

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The online version of this article (doi: 10.​1007/​s12471-016-0890-y) contains a movie, which is available to authorized users.
A 74-year-old male was analysed for exertional dyspnoea in our outpatient clinic. Cardiac MRI showed diffuse enhancing pericardial soft-tissue (Fig. 1a), but no late gadolinium myocardial enhancement. PET-CT showed the pericardial and peri-renal soft-tissue mass to be calcified, however without FDG uptake. Surgical biopsy of the omentum revealed amyloid depositions fitting with a diagnosis of systemic amyloid. Systemic therapy was started, but the heart failure was progressive and not responding to medical therapy. On repeat cardiac MRI flattening of the ventricular septum during expiration was seen during free breathing (online movie 1). Postmortem macroscopic examinations of the heart and kidney showed extensive peri-renal and pericardial amyloid deposition (Fig. 1b).
Cardiac amyloid is a rare systemic disease (6 per million cases). In most cases there is restrictive heart failure because of amyloid depositions. The usual pattern seen on cardiac MRI is late gadolinium enhancement of the entire subendocardial ring. It is very rare for systemic amyloid to present with pericardial amyloid depositions causing severe constrictive heart failure [13].

Conflict of interest

F.A.A. Mohamed Hoesein, M.J. Swaans, L.S. Jiwa, C.A. Seldenrijk and H.W. van Es declare that they have no competing interests.
Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
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Titel
An unusual case of systemic amyloid causing constrictive heart failure
verfasst von
F. A. A. Mohamed Hoesein
M. J. Swaans
L. S. Jiwa
C. A. Seldenrijk
H. W. van Es
Publikationsdatum
18.08.2016
Verlag
Bohn Stafleu van Loghum
Erschienen in
Netherlands Heart Journal / Ausgabe 9/2016
Print ISSN: 1568-5888
Elektronische ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-016-0890-y

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