open access

Vol 64, No 5 (2006)
Other
Published online: 2006-06-01
Submitted: 2012-12-28
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Case reports
Primary cardiac amyloidosis – condition which can be diagnosed by a cardiologist

Marzenna Zielińska, Włodzimierz Koniarek, Krzysztof Kaczmarek, Marek Maciejewski, Małgorzata Wągrowska-Danilewicz, Jan Henryk Goch
Kardiol Pol 2006;64(5):517-521.

open access

Vol 64, No 5 (2006)
Other
Published online: 2006-06-01
Submitted: 2012-12-28

Abstract

Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins. The disease symptoms are typical of multisystem failure. Common presenting features include nephrotic syndrome, hepatomegaly, sensomotor peripheral neuropathy and, in the case of cardiac involvement, congestive heart failure. This last sign appears very seldom as alone, without any others. Cardiac involvement generally denotes a poor prognosis, regardless of the method of treatment. The median survival rate from onset of congestive heart failure is 6 months. Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life. The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure. Because of lack of the other organ involvement symptoms, the correct diagnosis was made very late. The authors place emphasis on a simple diagnostic tool such as the correlation between the low voltage in the limb ECG leads and the echocardiographic sings of left ventricular hypertrophy. The combination of specific ECG, echocardiographic findings and positive extracardiac tissue biopsy may be sufficient to reach correct diagnosis. These examinations are easy accessible in non-specialist hospitals.

Abstract

Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins. The disease symptoms are typical of multisystem failure. Common presenting features include nephrotic syndrome, hepatomegaly, sensomotor peripheral neuropathy and, in the case of cardiac involvement, congestive heart failure. This last sign appears very seldom as alone, without any others. Cardiac involvement generally denotes a poor prognosis, regardless of the method of treatment. The median survival rate from onset of congestive heart failure is 6 months. Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life. The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure. Because of lack of the other organ involvement symptoms, the correct diagnosis was made very late. The authors place emphasis on a simple diagnostic tool such as the correlation between the low voltage in the limb ECG leads and the echocardiographic sings of left ventricular hypertrophy. The combination of specific ECG, echocardiographic findings and positive extracardiac tissue biopsy may be sufficient to reach correct diagnosis. These examinations are easy accessible in non-specialist hospitals.
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Keywords

primary amyloidosis; cardiac involvement; congestive heart failure

About this article
Title

Case reports
Primary cardiac amyloidosis – condition which can be diagnosed by a cardiologist

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Vol 64, No 5 (2006)

Pages

517-521

Published online

2006-06-01

Bibliographic record

Kardiol Pol 2006;64(5):517-521.

Keywords

primary amyloidosis
cardiac involvement
congestive heart failure

Authors

Marzenna Zielińska
Włodzimierz Koniarek
Krzysztof Kaczmarek
Marek Maciejewski
Małgorzata Wągrowska-Danilewicz
Jan Henryk Goch

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