Cardiac sarcoidosis with severe involvement of the right ventricle: a case report

Authors

  • Weverton César Siqueira Federal University of Minas Gerais. Faculty of Medicine. Internal Medicine Department
  • Samuel Gonçalves da Cruz Federal University of Minas Gerais. Faculty of Medicine. Internal Medicine Department
  • Angeliki Asimaki Harvard Medical School, Boston/MA. Beth Israel Deaconess Medical Center. Department of Pathology
  • Jeffrey Ern Saffitz Harvard Medical School, Boston/MA. Beth Israel Deaconess Medical Center. Department of Pathology
  • Maria da Consolação Vieira Moreira Federal University of Minas Gerais. Faculty of Medicine. Internal Medicine Department
  • Geraldo Brasileiro Filho Federal University of Minas Gerais. Faculty of Medicine. Pathology and Forensic Department
  • Luiz Otávio Savassi Rocha Federal University of Minas Gerais. Faculty of Medicine. Internal Medicine Department

DOI:

https://doi.org/10.4322/acr.2015.030

Keywords:

Sarcoidosis, Arrhythmogenic right ventricular cardiomyopathy, Immunohistochemistry, Heart diseases

Abstract

We present the case of a patient who underwent cardiac transplantation with the diagnosis of idiopathic dilated cardiomyopathy. Once the explanted heart was examined, a type of granulomatous myocarditis compatible with cardiac sarcoidosis was observed. However, there was severe involvement of the right ventricle, with markedly reduced width of the muscular layer and extensive fibrofatty replacement, findings similar to the ones encountered in cases of arrhythmogenic right ventricular cardiomyopathy (ARVC). Confocal immunofluorescence analysis revealed a reduced signal for plakoglobin and desmoplakin at the cardiac intercalated disks. The immunoreactive signal for desmin showed the typical sarcomeric distribution but not a concentrated signal at the intercalated disks, a pattern previously seen in an 11-year-old girl with Carvajal syndrome bearing a C-terminal truncating mutation in the desmoplakin gene. This case illustrates the difficult and challenging work involved in performing a differential diagnosis among idiopathic dilated cardiomyopathy, isolated cardiac sarcoidosis, and ARVC, all of which are clinical entities known to masquerade as one another

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Published

2015-12-10

How to Cite

Siqueira, W. C., Cruz, S. G. da, Asimaki, A., Saffitz, J. E., Moreira, M. da C. V., Brasileiro Filho, G., & Rocha, L. O. S. (2015). Cardiac sarcoidosis with severe involvement of the right ventricle: a case report. Autopsy and Case Reports, 5(4), 53-63. https://doi.org/10.4322/acr.2015.030

Issue

Section

Article / Clinical Case Report