Acute Myocarditis Associated With Desmosomal Gene Variants.

Gadolinium Heart Failure Humans Myocarditis / genetics Retrospective Studies Stroke Volume Troponin Ventricular Function, Left Young Adult

acute myocarditis cardiac magnetic resonance desmoplakin desmosomal gene variants prognosis

Journal

JACC. Heart failure

ISSN: 2213-1787

Titre abrégé: JACC Heart Fail

Pays: United States

ID NLM: 101598241

Informations de publication

Date de publication:
10 2022

Historique:

received: 29 04 2022

revised: 24 05 2022

accepted: 01 06 2022

entrez: 29 9 2022

pubmed: 30 9 2022

medline: 4 10 2022

Statut: ppublish

Résumé

The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown. The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV. In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[-]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up. In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(-) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM. Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk.

Sections du résumé

BACKGROUND

The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown.

OBJECTIVES

The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV.

METHODS

In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[-]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up.

RESULTS

In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(-) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM.

CONCLUSIONS

Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk.

Identifiants

DOI: 10.1016/j.jchf.2022.06.013 PMID: 36175056

pubmed: 36175056

pii: S2213-1779(22)00412-7

doi: 10.1016/j.jchf.2022.06.013

pii:

doi:

Substances chimiques

Troponin 0

Gadolinium AU0V1LM3JT

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

Pagination

714-727

Commentaires et corrections

Type : CommentIn

Informations de copyright

Déclaration de conflit d'intérêts

Funding Support and Author Disclosures Dr Ammirati has received a grant from the Italian Ministry of Health (GR-2019-12368506) and is a consultant for Kiniksa and Cytokinetics. Dr Adler is a consultant for Abbott, Abiomed, AstraZeneca, Endotronix, Ionis, Medtronic, and Novartis; is on the board of directors of Genstem Therapeutics; and is a shareholder of Rocket Pharmaceuticals. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.