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Int J Clin Exp Pathol 2013;6(12):2928-2935

Original Article
Clinical usefulness of immunohistochemistry for plakoglobin, N-cadherin, and connexin-
43 in the diagnosis of arrhythmogenic right ventricular cardiomyopathy

Yong-Seop Kwon, Tae In Park, Yongkeun Cho, Myung Hwan Bae, Sunzoo Kim

Department of Cardiology, Busan ST Mary’s Hospital, Busan, South Korea; Departments of Pathology, Cardiology, Kyungpook National
University Hospital, Kyungpook National University School of Medicine, Daegu, South Korea

Received October 1, 2013; Accepted October 29, 2013; Epub November 15, 2013; Published December 1, 2013

Abstract: Diagnosing arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging because no single diagnostic tool is
available to detect the disease. We evaluated whether analysis of plakoglobin, N-cadherin, and connexin-43 immunoreactivity can be used as a
significant test in diagnosis of ARVC. We selected subjects with suspicion of ARVC (n=22) in patients who underwent endomyocardial biopsy
(EMB) in Kyungpook National University Hospital (n=1326). The patients (n=22) were classified into definite ARVC patients (n=17) and
borderline ARVC (n=5). We selected control subjects (n=20) who were autopsied and died of non-cardiac disease. Hematoxylin-eosin, Masson’
s trichrome, and immunohistochemical stains for plakoglobin, N-cadherin, and connexin-43 were used for all specimens. Reduced
immunoreactivity of plakoglobin was observed in 13 (76%) of the 17 patients with a definite ARVC and in 4 (80%) of the 5 patients with a
borderline ARVC. All subjects displayed no significant reduction of the immunoreactivity for connexin-43 as well as for N-cadherin. Our
investigation revealed that the immunohistochemical analysis for plakoglobin had an accuracy of 81%, 76% sensitivity, and 84% specificity in
diagnosis of ARVC. Results of our study showed that the immunohistochemical analysis of plakoglobin had a relatively high sensitivity and
specificity in ARVC, but immunohistochemistry for plakoglobin alone could not be relied upon as a diagnostic test for ARVC. We confirmed that
N-cadherin and connexin-43 had no diagnostic value in ARVC. (IJCEP1310003).

Keywords: Plakoglobin, N-cadherin, connexin-43, arrhythmogenic right ventricular cardiomyopathy

Address correspondence to: Dr. Sunzoo Kim, Department of Pathology, Kyungpook National University Hospital, Kyungpook National
University School of Medicine, 50 Samduk-dong 2 Ga, Jung-gu, Daegu, South Korea, Zip code: 700-721. Tel: +82-53-420-5247; Fax: +82-53-
426-1525; E-mail: 94sunzoo@hanmail.net; 94sunzoo@hmail.knu.ac.kr