Abstract
Patients with dilated cardiomyopathy (DCM) sometimes show anti-mitochondrial M2 antibody
(AMA-M2) positivity. We aimed to compare the characteristics of DCM cases with and
without AMA-M2, and to describe cases of DCM with AMA-M2 positivity.
A total of 84 patients with DCM were analyzed. Six patients (7.1 %) were positive
for AMA-M2. Of these six patients, five (83.3 %) had primary biliary cirrhosis (PBC)
and four (66.7 %) had myositis. Patients with AMA-M2 positivity had more atrial fibrillation
and more premature ventricular contractions than those without. Left and right atrial
longitudinal dimensions were larger in patients with AMA positivity (left atrium,
65.9 mm vs. 54.7 mm, p = 0.02; right atrium, 57.0 mm vs. 46.1 mm, p = 0.02). Of the
six patients with AMA-M2 positivity, three underwent cardiac resynchronization therapy
with defibrillator implantation and three required catheter ablation treatment. Steroids
were used in three patients. One patient died of unresolved lethal arrhythmia and
another required re-hospitalization for heart failure; the remaining four patients
did not have adverse events.
Patients with DCM with AMA-M2 positivity had a higher affinity for PBC and myositis
than those without, and are characterized by atrial enlargement and arrhythmias.
Learning objective
Patients with dilated cardiomyopathy sometimes exhibit anti-mitochondrial M2 antibody
positivity. These patients are at higher risk for primary biliary cirrhosis and inflammatory
myositis, and their cardiac disorders are characterized by atrial enlargement and
various arrhythmias. The course of the disease up to the time of diagnosis and after
steroid use varies, and the prognosis is poor in advanced cases.
Keywords
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Article info
Publication history
Published online: March 07, 2023
Accepted:
February 22,
2023
Received in revised form:
February 18,
2023
Received:
September 22,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
