Diagnostic value of papillary muscle hypertrophy and mitral valve thickness to discriminate cardiac amyloidosis and Fabry disease.

Amyloidosis Echocardiography Fabry disease Mitral valve

Journal

International journal of cardiology
ISSN: 1874-1754
Titre abrégé: Int J Cardiol
Pays: Netherlands
ID NLM: 8200291

Informations de publication

Date de publication:
03 Dec 2023
Historique:
received: 29 06 2023
revised: 20 11 2023
accepted: 29 11 2023
pubmed: 5 12 2023
medline: 5 12 2023
entrez: 4 12 2023
Statut: aheadofprint

Résumé

Cardiac amyloidosis (CA) and Fabry disease (FD) cause myocardial damage but may also affect the valvular and subvalvular apparatus. We aimed to evaluate the diagnostic accuracy of new echocardiographic indices including mitral valve thickness and papillary muscle (PM) hypertrophy to differentiate CA and FD. In patients with confirmed CA and FD, a detailed assessment of valvular function, mitral valve leaflet thickness and PM area as well as PM left ventricular area ratio (PM/LV-ratio) was performed in offline analyses. Receiver operating characteristic curve analyses were conducted to determine the diagnostic accuracy of mitral valve thickness, PM hypertrophy, and PM/LV-ratio to distinguish CA from FD. We retrospectively analyzed a cohort of 129 patients (FD n = 49, CA n = 80). CA patients showed significantly more thickened mitral valve leaflets (4.1 ± 1.3 mm vs. 2.9 ± 1.1 mm, p < 0.001) and a higher PM area [4.0 (3.1-4.6) mm Our results suggest that the assessment of mitral valve thickness may be a new useful echocardiographic parameter to differentiate CA and FD, whereas papillary muscle hypertrophy and PM/LV-ratio showed a limited diagnostic performance to discriminate CA. German clinical trials registry: DRKS00027403.

Sections du résumé

BACKGROUND BACKGROUND
Cardiac amyloidosis (CA) and Fabry disease (FD) cause myocardial damage but may also affect the valvular and subvalvular apparatus. We aimed to evaluate the diagnostic accuracy of new echocardiographic indices including mitral valve thickness and papillary muscle (PM) hypertrophy to differentiate CA and FD.
METHODS METHODS
In patients with confirmed CA and FD, a detailed assessment of valvular function, mitral valve leaflet thickness and PM area as well as PM left ventricular area ratio (PM/LV-ratio) was performed in offline analyses. Receiver operating characteristic curve analyses were conducted to determine the diagnostic accuracy of mitral valve thickness, PM hypertrophy, and PM/LV-ratio to distinguish CA from FD.
RESULTS RESULTS
We retrospectively analyzed a cohort of 129 patients (FD n = 49, CA n = 80). CA patients showed significantly more thickened mitral valve leaflets (4.1 ± 1.3 mm vs. 2.9 ± 1.1 mm, p < 0.001) and a higher PM area [4.0 (3.1-4.6) mm
CONCLUSION CONCLUSIONS
Our results suggest that the assessment of mitral valve thickness may be a new useful echocardiographic parameter to differentiate CA and FD, whereas papillary muscle hypertrophy and PM/LV-ratio showed a limited diagnostic performance to discriminate CA. German clinical trials registry: DRKS00027403.

Identifiants

DOI: 10.1016/j.ijcard.2023.131629 PMID: 38048880
pubmed: 38048880
pii: S0167-5273(23)01763-1
doi: 10.1016/j.ijcard.2023.131629
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

131629

Informations de copyright

Copyright © 2023 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest Isabel Mattig is participant in the BIH Charité Clinician Scientist Program funded by the Charité – Universitätsmedizin Berlin and the Berlin Institute of Health at Charité (BIH). She received research grants from Pfizer Pharmaceuticals and Sanofi, lecture fee and financial reimbursement for advisory board activities from Sanofi outside the submitted work. SSp achieved financial reimbursement for lectures from Pfizer Pharmaceuticals. KH achieved financial reimbursement for consulting, advisory board activities and travel support by Akcea Therapeuticals Inc., Alnylam Pharmaceuticals Inc., Swedish Orphan Biovitrum, and Pfizer Pharmaceuticals, research funding by Alnylam Pharmaceuticals Inc., and Pfizer Pharmaceuticals as well as research funding by the foundation Charité (BIH clinical fellow). AB received lecture fee from Pfizer Pharmaceuticals.

Auteurs

Isabel Mattig (I)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, Berlin, Germany.

Tilman Steudel (T)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany.

Gina Barzen (G)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany.

David Frumkin (D)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany.

Sebastian Spethmann (S)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany.

Elena Romero Dorta (ER)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany.

Karl Stangl (K)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany.

Bettina Heidecker (B)

Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Benjamin Franklin, Berlin, Germany.

Ulf Landmesser (U)

Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Benjamin Franklin, Berlin, Germany.

Fabian Knebel (F)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, Berlin, Germany; Sana Klinikum Lichtenberg, Innere Medizin II: Schwerpunkt Kardiologie, Berlin, Germany.

Sima Canaan-Kühl (S)

Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Medizinische Klinik mit Schwerpunkt Nephrologie und Internistische Intensivmedizin, Fabry Zentrum, Zentrum für seltene Nierenerkrankungen (CeRKiD), Campus Charité Mitte, Berlin, Germany.

Katrin Hahn (K)

Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Klinik für Neurologie und Experimentelle Neurologie, Berlin, Germany.

Anna Brand (A)

Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Charité Mitte, Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117 Berlin, Germany; Amyloidosis Center Charité Berlin (ACCB), Charité - Universitätsmedizin Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany; Deutsches Herzzentrum der Charité, Department of Cardiology, Angiology and Intensive Care Medicine, Campus Benjamin Franklin, Berlin, Germany. Electronic address: anna.brand@dhzc-charite.de.

Classifications MeSH