AIMS: To provide a standardized endomyocardial biopsy (EMB) protocol and diagnostic quantitative parameters for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). The Task Force criteria for the in vivo diagnosis of ARVC/D include tissue characterization by EMB as a major criterion. METHODS AND RESULTS: EMBs were simulated in vitro with a Cordis bioptome in explanted hearts from six groups: diffuse (n = 10) and segmental (n = 10) ARVC/D, dilated cardiomyopathy (DC) (n = 10), controls (n = 10), adipositas cordis (n = 10), elderly >80 years (n = 10). Sampling sites were the RV inferior-subtricuspid, antero-apical, and mid-outflow tract (RVOT), the septum, and the left ventricle (LV). Histomorphometry was performed to evaluate the amount of myocardium and fibrous and fatty tissues. Myocyte diameters and abnormalities were also assessed. By selecting a 95% specificity, the ARVC/D diagnostic cut-offs on cumulative RV EMB samples are myocardium <59%, fibrosis >31% and fat >22% (80, 50, and 50% sensitivity, respectively). By excluding elderly and obese people groups a lower cut-off for fat was found (>9%). A high variability between different RV sampling sites was observed; the antero-apical was the most informative region although fat at this level is non-specific. No useful diagnostic cut-off for fatty tissue was identified at the antero-apical and RVOT area. No significant difference was found for any tissue parameter either in septal or in LV EMB. Increased RV myocyte diameters and cytological changes were detected in ARVC/D and DC. CONCLUSION: The residual myocardium is the main diagnostic morphometric parameter in ARVC/D, whereas fat at the apex is non-specific. Sensitivity and specificity vary according to the RV region. Target sampling of the triangle of dysplasia is required, although only a single region is often informative, emphasizing the usefulness of imaging-guided EMB. There is no diagnostic value of either septal or LV EMB. Cardiomyopathic changes of the myocytes also appear important for establishing a pathological diagnosis.
AIMS: To provide a standardized endomyocardial biopsy (EMB) protocol and diagnostic quantitative parameters for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). The Task Force criteria for the in vivo diagnosis of ARVC/D include tissue characterization by EMB as a major criterion. METHODS AND RESULTS: EMBs were simulated in vitro with a Cordis bioptome in explanted hearts from six groups: diffuse (n = 10) and segmental (n = 10) ARVC/D, dilated cardiomyopathy (DC) (n = 10), controls (n = 10), adipositas cordis (n = 10), elderly >80 years (n = 10). Sampling sites were the RV inferior-subtricuspid, antero-apical, and mid-outflow tract (RVOT), the septum, and the left ventricle (LV). Histomorphometry was performed to evaluate the amount of myocardium and fibrous and fatty tissues. Myocyte diameters and abnormalities were also assessed. By selecting a 95% specificity, the ARVC/D diagnostic cut-offs on cumulative RV EMB samples are myocardium <59%, fibrosis >31% and fat >22% (80, 50, and 50% sensitivity, respectively). By excluding elderly and obese people groups a lower cut-off for fat was found (>9%). A high variability between different RV sampling sites was observed; the antero-apical was the most informative region although fat at this level is non-specific. No useful diagnostic cut-off for fatty tissue was identified at the antero-apical and RVOT area. No significant difference was found for any tissue parameter either in septal or in LV EMB. Increased RV myocyte diameters and cytological changes were detected in ARVC/D and DC. CONCLUSION: The residual myocardium is the main diagnostic morphometric parameter in ARVC/D, whereas fat at the apex is non-specific. Sensitivity and specificity vary according to the RV region. Target sampling of the triangle of dysplasia is required, although only a single region is often informative, emphasizing the usefulness of imaging-guided EMB. There is no diagnostic value of either septal or LV EMB. Cardiomyopathic changes of the myocytes also appear important for establishing a pathological diagnosis.
Authors: Mireia Alcalde; Oscar Campuzano; Georgia Sarquella-Brugada; Elena Arbelo; Catarina Allegue; Sara Partemi; Anna Iglesias; Antonio Oliva; Josep Brugada; Ramon Brugada Journal: Clin Res Cardiol Date: 2014-11-15 Impact factor: 5.460
Authors: Frank I Marcus; William J McKenna; Duane Sherrill; Cristina Basso; Barbara Bauce; David A Bluemke; Hugh Calkins; Domenico Corrado; Moniek G P J Cox; James P Daubert; Guy Fontaine; Kathleen Gear; Richard Hauer; Andrea Nava; Michael H Picard; Nikos Protonotarios; Jeffrey E Saffitz; Danita M Yoerger Sanborn; Jonathan S Steinberg; Harikrishna Tandri; Gaetano Thiene; Jeffrey A Towbin; Adalena Tsatsopoulou; Thomas Wichter; Wojciech Zareba Journal: Circulation Date: 2010-02-19 Impact factor: 29.690
Authors: Frank I Marcus; Wojciech Zareba; Hugh Calkins; Jeffrey A Towbin; Cristina Basso; David A Bluemke; N A Mark Estes; Michael H Picard; Danita Sanborn; Gaetano Thiene; Thomas Wichter; David Cannom; David J Wilber; Melvin Scheinman; Henry Duff; James Daubert; Mario Talajic; Andrew Krahn; Michael Sweeney; Hasan Garan; Scott Sakaguchi; Bruce B Lerman; Charles Kerr; Jack Kron; Jonathan S Steinberg; Duane Sherrill; Kathleen Gear; Mary Brown; Patricia Severski; Slava Polonsky; Scott McNitt Journal: Heart Rhythm Date: 2009-03-11 Impact factor: 6.343
Authors: Frank I Marcus; William J McKenna; Duane Sherrill; Cristina Basso; Barbara Bauce; David A Bluemke; Hugh Calkins; Domenico Corrado; Moniek G P J Cox; James P Daubert; Guy Fontaine; Kathleen Gear; Richard Hauer; Andrea Nava; Michael H Picard; Nikos Protonotarios; Jeffrey E Saffitz; Danita M Yoerger Sanborn; Jonathan S Steinberg; Harikrishna Tandri; Gaetano Thiene; Jeffrey A Towbin; Adalena Tsatsopoulou; Thomas Wichter; Wojciech Zareba Journal: Eur Heart J Date: 2010-02-19 Impact factor: 29.983
Authors: Maria E Campian; Hein J Verberne; Maxim Hardziyenka; Elisabeth A A de Groot; Astrid F van Moerkerken; Berthe L F van Eck-Smit; Hanno L Tan Journal: Eur J Nucl Med Mol Imaging Date: 2010-07-06 Impact factor: 9.236
Authors: Kalliopi Pilichou; Carol Ann Remme; Cristina Basso; Maria E Campian; Stefania Rizzo; Phil Barnett; Brendon P Scicluna; Barbara Bauce; Maurice J B van den Hoff; Jacques M T de Bakker; Hanno L Tan; Marialuisa Valente; Andrea Nava; Arthur A M Wilde; Antoon F M Moorman; Gaetano Thiene; Connie R Bezzina Journal: J Exp Med Date: 2009-07-27 Impact factor: 14.307