2008
DOI: 10.1093/eurheartj/ehn415
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Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria

Abstract: 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.

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Cited by 164 publications
(99 citation statements)
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“…It is surprising that arrhythmogenic RV cardiomyopathy/dysplasia, a disorder that primarily affects the RV "triangle of dysplasia," was commonly diagnosed on LV EMB. 18 These findings extend a smaller comparison study of LV and RV biopsy that found biventricular EMB has a superior diagnostic yield compared with RV EMB. 19 The strongest conclusion from these data and the other recently published LV EMB case series is that the risk of major complications from LV EMB is low (<1%) when performed by experienced operators at centers with appropriate infrastructure support.…”
Section: Article See P 1531supporting
confidence: 77%
“…It is surprising that arrhythmogenic RV cardiomyopathy/dysplasia, a disorder that primarily affects the RV "triangle of dysplasia," was commonly diagnosed on LV EMB. 18 These findings extend a smaller comparison study of LV and RV biopsy that found biventricular EMB has a superior diagnostic yield compared with RV EMB. 19 The strongest conclusion from these data and the other recently published LV EMB case series is that the risk of major complications from LV EMB is low (<1%) when performed by experienced operators at centers with appropriate infrastructure support.…”
Section: Article See P 1531supporting
confidence: 77%
“…Distinguishing between athlete's heart and hypertrophic or arrhythmogenic cardiomyopathy can be very challenging despite modern imaging 20 and this uncertainty may be incompletely resolved at autopsy. For example, fibro-fatty infiltration of the right ventricle may not be as specific for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as has previously been thought, 21 whilst hypertrophic cardiomyopathy and athlete's heart may also be indistinguishable on histology.…”
Section: Inherited Versus Acquired Heart Disease-not Black and White mentioning
confidence: 98%
“…[6][7][8][9][10][11] Our first patient had his genetic screening for mutations of desmosomal proteins, also his father had the same diagnosis, and the third patient had a family history of ARVD/C, and his elder brother managed by medical therapy.…”
Section: Discussionmentioning
confidence: 99%