Clinical characteristics and outcome of biopsy-proven myocarditis in children - Results of the German prospective multicentre registry "MYKKE".


Journal

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

Informations de publication

Date de publication:
15 06 2022
Historique:
received: 22 09 2021
revised: 08 02 2022
accepted: 11 03 2022
pubmed: 20 3 2022
medline: 4 5 2022
entrez: 19 3 2022
Statut: ppublish

Résumé

Heart failure (HF) due to myocarditis might not respond in the same way to standard therapy as HF due to other aetiologies. The aim of this study was to investigate the value of endomyocardial biopsies (EMB) for clinical decision-making and its relation to the outcome of paediatric patients with myocarditis. Clinical and EMB data of children with myocarditis collected for the MYKKE-registry between 2013 and 2020 from 23 centres were analysed. EMB studies included histology, immunohistology, and molecular pathology. The occurrence of major adverse cardiac events (MACE) including mechanical circulatory support (MCS), heart transplantation, and/or death was defined as a combined endpoint. Myocarditis was diagnosed in 209/260 patients: 64% healing/chronic lymphocytic myocarditis, 23% acute lymphocytic myocarditis (AM), 14% healed myocarditis, no giant cell myocarditis. The median age was 12.8 (1.4-15.9) years. Time from symptom-onset to EMB was 11.0 (4.0-29.0) days. Children with AM and high amounts of mononuclear cell infiltrates were significantly younger with signs of HF compared to those with healing/chronic or healed myocarditis. Myocardial viral DNA/RNA detection had no significant effect on outcome. The worst event-free survival was seen in patients with healing/chronic myocarditis (24%), followed by acute (31%) and healed myocarditis (58%, p = 0.294). A weaning rate of 64% from MCS was found in AM. EMB provides important information on the type and stage of myocardial inflammation and supports further decision-making. Children with fulminant clinical presentation, high amounts of mononuclear cell infiltrates or healing/chronic inflammation and young age have the highest risk for MACE.

Sections du résumé

BACKGROUND
Heart failure (HF) due to myocarditis might not respond in the same way to standard therapy as HF due to other aetiologies. The aim of this study was to investigate the value of endomyocardial biopsies (EMB) for clinical decision-making and its relation to the outcome of paediatric patients with myocarditis.
METHODS
Clinical and EMB data of children with myocarditis collected for the MYKKE-registry between 2013 and 2020 from 23 centres were analysed. EMB studies included histology, immunohistology, and molecular pathology. The occurrence of major adverse cardiac events (MACE) including mechanical circulatory support (MCS), heart transplantation, and/or death was defined as a combined endpoint.
RESULTS
Myocarditis was diagnosed in 209/260 patients: 64% healing/chronic lymphocytic myocarditis, 23% acute lymphocytic myocarditis (AM), 14% healed myocarditis, no giant cell myocarditis. The median age was 12.8 (1.4-15.9) years. Time from symptom-onset to EMB was 11.0 (4.0-29.0) days. Children with AM and high amounts of mononuclear cell infiltrates were significantly younger with signs of HF compared to those with healing/chronic or healed myocarditis. Myocardial viral DNA/RNA detection had no significant effect on outcome. The worst event-free survival was seen in patients with healing/chronic myocarditis (24%), followed by acute (31%) and healed myocarditis (58%, p = 0.294). A weaning rate of 64% from MCS was found in AM.
CONCLUSIONS
EMB provides important information on the type and stage of myocardial inflammation and supports further decision-making. Children with fulminant clinical presentation, high amounts of mononuclear cell infiltrates or healing/chronic inflammation and young age have the highest risk for MACE.

Identifiants

pubmed: 35304189
pii: S0167-5273(22)00387-4
doi: 10.1016/j.ijcard.2022.03.026
pii:
doi:

Types de publication

Journal Article Multicenter Study

Langues

eng

Sous-ensembles de citation

IM

Pagination

95-104

Informations de copyright

Copyright © 2022 Elsevier B.V. All rights reserved.

Auteurs

Franziska Seidel (F)

German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Berlin, Germany; Charité-Universitätsmedizin Berlin, Department of Pediatric Cardiology, Berlin, Germany; Experimental and Clinical Research Center, a cooperation between the Max-Delbrück-Center for Molecular Medicine in the Helmholtz Association and the Charité-Universitätsmedizin Berlin, Germany; Charité-Universitätsmedizin Berlin, Institute for Imaging Science and Computational Modelling in Cardiovascular Medicine, Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany. Electronic address: seidel@dhzb.de.

Bernd Opgen-Rhein (B)

Charité-Universitätsmedizin Berlin, Department of Pediatric Cardiology, Berlin, Germany.

Axel Rentzsch (A)

Department for Paediatric Cardiology, Saarland University Medical Center, Homburg, Germany.

Martin Boehne (M)

Department of Paediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.

Bardo Wannenmacher (B)

Clinic for Paediatric Cardiology, Heart Centre, University of Leipzig, Leipzig, Germany.

Dorotheé Boecker (D)

Department for Paediatric Cardiology, University Hospital Erlangen, Erlangen, Germany.

Katja Reineker (K)

Department for Paediatric Cardiology, University Heart Center Freiburg, Freiburg, Germany.

Maria Grafmann (M)

Department for Paediatric Cardiology, University Heart & Vascular Center Hamburg, Hamburg, Germany.

Gesa Wiegand (G)

Department for Paediatric Cardiology, University Hospital Tübingen, Tübingen, Germany.

Tobias Hecht (T)

Center for Congenital Heart Disease/Pediatric Cardiology, Heart- and Diabetescenter NRW, University Clinic of Ruhr University Bochum, Bad Oeynhausen, Germany.

Daniela Kiski (D)

Department for Paediatric Cardiology, University Hospital Münster, Münster, Germany.

Marcus Fischer (M)

Department of Paediatric Cardiology and Paediatric Intensive Care, Ludwig Maximilians University of Munich, Munich, Germany.

Konstantin Papakostas (K)

Department for Paediatric Cardiology, Klinikum Links der Weser, Bremen, Germany.

Bettina Ruf (B)

Department for Paediatric Cardiology, German Heart Centre Munich, Munich, Germany.

Jennifer Kramp (J)

Department for Paediatric Cardiology, University Hospital Cologne, Cologne, Germany.

Marcus Khalil (M)

Department for Paediatric Cardiology, University Hospital Giessen, Giessen, Germany.

Michael Kaestner (M)

Pediatric Cardiology, University Hospital Ulm, Ulm, Germany.

Michael Steinmetz (M)

Department for Paediatric Cardiology, Universitätsmedizin Göttingen, Göttingen, Germany.

Gunther Fischer (G)

Department for Paediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.

Sevinc Özcan (S)

Pediatric Cardiology, University Hospital RWTH Aachen, Aachen, Germany.

Noa Freudenthal (N)

Pediatric Cardiology, University Hospital Bonn, Bonn, Germany.

Ulrich Schweigmann (U)

Pediatric Cardiology, Olgahospital, Stuttgart, Germany.

Regina Hellwig (R)

Pediatric Cardiology and Congenital Heart Defects, Center for Pediatrics, University Hospital Heidelberg, Heidelberg. Germany.

Thomas Pickardt (T)

Competence Network for Congenital Heart Defects, Berlin, Germany.

Karin Klingel (K)

Cardiopathology, Institute for Pathology and Neuropathology, University Hospital Tübingen, Tübingen, Germany.

Daniel Messroghli (D)

DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany; Internal Medicine-Cardiology, German Heart Center, Germany; Internal Medicine and Cardiology, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Stephan Schubert (S)

German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin, Germany; Center for Congenital Heart Disease/Pediatric Cardiology, Heart- and Diabetescenter NRW, University Clinic of Ruhr University Bochum, Bad Oeynhausen, Germany.

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