title: Clinical usefulness of immunohistochemistry for plakoglobin, N-cadherin, and connexin-43 in the diagnosis of arrhythmogenic right ventricular cardiomyopathy.
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.
authors.affiliation: Department of Cardiology, Busan ST Mary's Hospital Busan, South Korea.
authors.forename: Yong-Seop
authors.initials: YS
authors.lastname: Kwon
authors.forename: Tae In
authors.initials: TI
authors.lastname: Park
authors.forename: Yongkeun
authors.initials: Y
authors.lastname: Cho
authors.forename: Myung Hwan
authors.initials: MH
authors.lastname: Bae
authors.forename: Sunzoo
authors.initials: S
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000032:analysis*; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000378:metabolism; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D015820:Cadherins / Q000032:analysis*; D016022:Case-Control Studies; D018031:Connexin 43 / Q000032:analysis*; D051180:Desmoplakins / Q000032:analysis*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000737:chemistry* / Q000473:pathology; D011237:Predictive Value of Tests; D055815:Young Adult; D051185:gamma Catenin
chemical_list: D015703:Antigens, CD; D015415:Biomarkers; C050361:CDH2 protein, human; D015820:Cadherins; D018031:Connexin 43; D051180:Desmoplakins; C500216:GJA1 protein, human; C495292:JUP protein, human; D051185:gamma Catenin
keywords: N-cadherin; Plakoglobin; arrhythmogenic right ventricular cardiomyopathy; connexin-43