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Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report

INTRODUCTION: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibr...

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Autores principales: Fabbian, Fabio, Stabellini, Nevio, Sartori, Sergio, Tombesi, Paola, Aleotti, Arrigo, Bergami, Maurizio, Uggeri, Simona, Galdi, Adriana, Molino, Christian, Catizone, Luigi
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2254633/
https://www.ncbi.nlm.nih.gov/pubmed/18163912
http://dx.doi.org/10.1186/1752-1947-1-187
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author Fabbian, Fabio
Stabellini, Nevio
Sartori, Sergio
Tombesi, Paola
Aleotti, Arrigo
Bergami, Maurizio
Uggeri, Simona
Galdi, Adriana
Molino, Christian
Catizone, Luigi
author_facet Fabbian, Fabio
Stabellini, Nevio
Sartori, Sergio
Tombesi, Paola
Aleotti, Arrigo
Bergami, Maurizio
Uggeri, Simona
Galdi, Adriana
Molino, Christian
Catizone, Luigi
author_sort Fabbian, Fabio
collection PubMed
description INTRODUCTION: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation. CASE PRESENTATION: A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications. DISCUSSION: Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.
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spelling pubmed-22546332008-02-27 Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report Fabbian, Fabio Stabellini, Nevio Sartori, Sergio Tombesi, Paola Aleotti, Arrigo Bergami, Maurizio Uggeri, Simona Galdi, Adriana Molino, Christian Catizone, Luigi J Med Case Reports Case Report INTRODUCTION: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation. CASE PRESENTATION: A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications. DISCUSSION: Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD. BioMed Central 2007-12-29 /pmc/articles/PMC2254633/ /pubmed/18163912 http://dx.doi.org/10.1186/1752-1947-1-187 Text en Copyright © 2007 Fabbian et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Fabbian, Fabio
Stabellini, Nevio
Sartori, Sergio
Tombesi, Paola
Aleotti, Arrigo
Bergami, Maurizio
Uggeri, Simona
Galdi, Adriana
Molino, Christian
Catizone, Luigi
Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title_full Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title_fullStr Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title_full_unstemmed Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title_short Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
title_sort light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2254633/
https://www.ncbi.nlm.nih.gov/pubmed/18163912
http://dx.doi.org/10.1186/1752-1947-1-187
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