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• There may also be an overlap with idiopathic giant cell myocarditis
  Oscar,M Jolobe
  Published on: 4 February 2025

• Published on: 4 February 2025

  There may also be an overlap with idiopathic giant cell myocarditis

  • Oscar,M Jolobe, retired geriatrician British Medical Association

  Due to the considerable degree of overlap between stigmata of cardiac sarcoidosis(CS) and idiopathic giant cell myocarditis(IGCM)[1], an evaluation of the association of CS and arrhythmogenic cardiomyopathy(ACM)[2] should be accompanied by a caveat acknowledging the potential for an association along the same lines also to exist between ACM and IGCM. The evidence which supports such an association is to be found in the study which showed a reduction in immunoreactive signal for the desmosomal protein plakoglobin at the cardiac myocyte junctions not only in patients with CS but, also, in patients with IGCM[3].
  The following are some of the parallels between CS, IGCM, and ACM:-
  (i)The atrioventricular(AV) conduction derangements which characterise the association of CS and ACM[2] have , as their counterpart, the AV conduction defects documented in CS and in IGCM, respectively[4], and also in ACM, on its own.{5].
  (ii)Interventricular septum involvement documented in some of the patients with the association of CS and ACM[2] has, as its counterpart, interventricular septal infiltration by IGCM[6], and by ACM[7].
  (iii)Finally, extensive myocardial fibrosis, sometimes associated with concomitant replacement of myocardium by adipocytes, may be a feature of CS[8]. It may also be a feature of IGCM(without concomitant adipocyte infiltration)[9], and may be a feature of ACM even in the absence of concomitant adipocyte infiltration[10].
  For all t...

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  Due to the considerable degree of overlap between stigmata of cardiac sarcoidosis(CS) and idiopathic giant cell myocarditis(IGCM)[1], an evaluation of the association of CS and arrhythmogenic cardiomyopathy(ACM)[2] should be accompanied by a caveat acknowledging the potential for an association along the same lines also to exist between ACM and IGCM. The evidence which supports such an association is to be found in the study which showed a reduction in immunoreactive signal for the desmosomal protein plakoglobin at the cardiac myocyte junctions not only in patients with CS but, also, in patients with IGCM[3].
  The following are some of the parallels between CS, IGCM, and ACM:-
  (i)The atrioventricular(AV) conduction derangements which characterise the association of CS and ACM[2] have , as their counterpart, the AV conduction defects documented in CS and in IGCM, respectively[4], and also in ACM, on its own.{5].
  (ii)Interventricular septum involvement documented in some of the patients with the association of CS and ACM[2] has, as its counterpart, interventricular septal infiltration by IGCM[6], and by ACM[7].
  (iii)Finally, extensive myocardial fibrosis, sometimes associated with concomitant replacement of myocardium by adipocytes, may be a feature of CS[8]. It may also be a feature of IGCM(without concomitant adipocyte infiltration)[9], and may be a feature of ACM even in the absence of concomitant adipocyte infiltration[10].
  For all those reasons a worthwhile follow up of the study by Asimaki et al would be an exploration of the relationship between ACM and IGCM along the same lines and using the same methodology as the exploration of the relationship between CS and ACM[2].
  References
  [1]Birnie DH., Nair V., Veinot JP
  Cardiac sarcoidosis and giant cell myocarditis: Actually, 2 ends of the same disease?
  J Am Heart Assoc 2021;10:e020542
  [2]Rossi AV., Palazzini M., Ammirati E rt al
  Coexistence of cardiac sarcoidosis and arrhythmogenic cardiomyopathy-associated genetic variants: a multicentre case-control study
  Heart 2025 doi:10.1136/heartjnl-2024-324525
  [3]Asimaki A., Tandri H., Duffy ER et al
  Altered desmosomal proteins in granulomatous myocarditis and potential pathogenic links to arryhthmogenic right ventricular cardiomyopathy
  Circ Arrhythm Electrophysiol 2011;4:743-752
  [4]Kandolin R., Lehtonen J., Kupari M
  Cardiac sarcoidosis and giant cell myocarditis as causes of atrioventricular block in young and middle aged adults
  Circ Arrhythm Electrophysiol 2011;4:303-309
  [5]Liang E., Wu L., Fan S et al
  Bradyarrhythmias in arrhythmogenic right ventricular cardiomyopathy
  Am J Cardiol 2019;123:1690-1695
  [6]Iturriagagotia A., Meert V., De Cocker J et al
  progressive thinning of the basal interventricular septum by giant cell myocarditis
  JACC Case Reports 2020;2:180-185
  [7]Noorman M., Groeneweg JA., Asimaki A et al
  End stage arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum
  Heart Rhythm 2013;10:283-289
  [8]Petrovic M., Buja LM., Kar B et al
  Cardiac sarcoidosis presenting as arrhythmogenic right ventricular cardiomyopathy/dysplasia with ventricular aneurysms: a case report
  Cardiovascular Pathology 2018;33:1-5
  [9]Yokoyama H., Yamaguchi M., Tobita K., Saito S
  Different reverse remodeling between left ventricle and right ventricle in fulminating heart failure due to giant cell myocarditis: acas report
  Eur Heart J Case Reports 2021;S(8): 1-7
  [10]Navarro-Manchon J., Fernandez E., Igual B et al
  Left dominant arrhythmogenic cardiomyopathy caused by a novel nonsense mutation in desmoplakin
  Rev esp Cardiol 2011;64:S30-S34

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  Conflict of Interest:
  None declared.

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