{"id":9552,"date":"2021-12-23T19:45:02","date_gmt":"2021-12-23T19:45:02","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=9552"},"modified":"2025-12-30T20:29:05","modified_gmt":"2025-12-30T20:29:05","slug":"arrhythmogenic-cardiomyopathy","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/arrhythmogenic-cardiomyopathy\/","title":{"rendered":"New Diagnostic Criteria for Arrhythmogenic Cardiomyopathy"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>These new insights into the heterogeneous genetic mutations and phenotypic manifestations of ACM led to a critical revision of the 2010 ITF criteria, which exclusively targeted RV classical forms and did not include the tissue characterization by contrast enhanced cardiac magnetic resonance (CMR) imaging. Accordingly, an International Expert consensus document has been recently developed to provide upgraded criteria (\u201cthe Padua Criteria\u201d) for the diagnosis of the whole spectrum of ACM phenotypes. <!--more--><\/p>\n<p><strong>\u201cPadua criteria\u201d for diagnosis of arrhythmogenic cardiomyopathy<\/strong><\/p>\n<table style=\"border-collapse: collapse; width: 100%;\" border=\"1\">\n<tbody>\n<tr>\n<td style=\"width: 21.4896%;\"><strong>Category<\/strong><\/td>\n<td style=\"width: 38.0952%;\"><strong>Right Ventricle<\/strong><\/td>\n<td style=\"width: 40.4151%;\"><strong>Left Ventricle<\/strong><\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">I. Morpho-functional ventricular abnormalities<\/td>\n<td style=\"width: 38.0952%;\">By echocardiography, CMR or angiography:<br \/>\nMajor<br \/>\n\u2022 Regional RV akinesia, dyskinesia, or bulging, plus, one of the following:<br \/>\n&#8211; global RV dilatation (increase of RV EDV according to the imaging test specific nomograms)<br \/>\n&#8211; global RV systolic dysfunction (reduction of RV ejection fraction (EF) according to the imaging test specific nomograms)<br \/>\nMinor<br \/>\n\u2022 Regional RV akinesia, dyskinesia, or aneurysm of RV free wall<\/td>\n<td style=\"width: 40.4151%;\">By echocardiography, CMR or angiography:<br \/>\nMinor<br \/>\n\u2022 Global LV systolic dysfunction (depression of LV EF or reduction of echocardiographic global longitudinal strain), with or without LV dilatation (increase of LV EDV according to the imaging test specific nomograms for age, sex, and body surface area (BSA))<br \/>\nMinor<br \/>\n\u2022 Regional LV hypokinesia or akinesia of LV free wall, septum, or both<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">II. Structural myocardial abnormalities<\/td>\n<td style=\"width: 38.0952%;\">By CE-CMR:<br \/>\nMajor<br \/>\n\u2022 Transmural LGE (stria pattern) of \u22651 RV region(s) (inlet, outlet, and apex in 2 orthogonal views)<br \/>\nBy EMB (limited indications):<br \/>\nMajor<br \/>\n\u2022 Fibrous replacement of the myocardium in \u22651 sample, with or without fatty tissue<\/td>\n<td style=\"width: 40.4151%;\">By CE-CMR:<br \/>\nMajor<br \/>\n\u2022 LV LGE (stria pattern) of \u22651 Bull\u2019s Eye segment(s) (in 2\u00a0orthogonal views) of the free wall (subepicardial or\u00a0midmyocardial), septum, or both (excluding septal\u00a0junctional LGE<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">III. Repolarization abnormalities<\/td>\n<td style=\"width: 38.0952%;\">Major<br \/>\n\u2022 Inverted T waves in right precordial leads (V1, V2, and V3) or\u00a0beyond in individuals with complete pubertal development (in\u00a0the absence of complete RBBB)<br \/>\nMinor<br \/>\n\u2022 Inverted T waves in leads V1 and V2 in individuals with\u00a0completed pubertal development (in the absence of complete\u00a0RBBB)<br \/>\n\u2022 Inverted T waves in V1, V2, V3 and V4 in individuals with\u00a0completed pubertal development in the presence of\u00a0complete RBBB.<\/td>\n<td style=\"width: 40.4151%;\">Minor<br \/>\nInverted T waves in left precordial leads (V 4\u2013V 6) (in the absence of complete LBBB)<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">IV. Depolarization abnormalities<\/td>\n<td style=\"width: 38.0952%;\">Minor<br \/>\n\u2022 Epsilon wave (reproducible low amplitude signals between end\u00a0of QRS complex to onset of the T wave) in the right precordial\u00a0leads (V1 to V3)<br \/>\n\u2022 Terminal activation duration of QRS \u2265 55 ms measured from the\u00a0nadir of the S wave to the end of the QRS, including R\u2019, in V1, V2,\u00a0or V3 (in the absence of complete RBBB)<\/td>\n<td style=\"width: 40.4151%;\">Minor<br \/>\n\u2022 Low QRS voltages (&lt;0.5 mV peak to peak) in limb leads (in the\u00a0absence of obesity, emphysema, or pericardial effusion)<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">V. Ventricular arrhythmias<\/td>\n<td style=\"width: 38.0952%;\">Major<br \/>\n\u2022 Frequent ventricular extrasystoles (&gt;500 per 24 h), non-sustained\u00a0or sustained ventricular tachycardia of LBBB morphology<br \/>\nMinor<br \/>\n\u2022 Frequent ventricular extrasystoles (&gt;500 per 24 h), non-sustained\u00a0or sustained ventricular tachycardia of LBBB morphology with\u00a0inferior axis (\u201cRVOT pattern\u201d)<\/td>\n<td style=\"width: 40.4151%;\">Minor<br \/>\n\u2022 Frequent ventricular extrasystoles (&gt;500 per 24 h), non-sustained\u00a0or sustained ventricular tachycardia with a RBBB morphology\u00a0(excluding the \u201cfascicular pattern\u201d)<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 21.4896%;\">VI. Family history\/genetics<\/td>\n<td style=\"width: 38.0952%;\" colspan=\"2\">Major<br \/>\n\u2022 ACM confirmed in a first-degree relative who meets diagnostic criteria<br \/>\n\u2022 ACM confirmed pathologically at autopsy or surgery in a first degree relative<br \/>\n\u2022 Identification of a pathogenic or likely pathogenetic ACM mutation in the patient under evaluation<br \/>\nMinor<br \/>\n\u2022 History of ACM in a first-degree relative in whom it is not possible or practical to determine whether the family member meets\u00a0diagnostic criteria<br \/>\n\u2022 Premature sudden death (&lt;35 years of age) due to suspected ACM in a first-degree relative<br \/>\n\u2022 ACM confirmed pathologically or by diagnostic criteria in a second-degree relative<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>ACM = arrhythmogenic cardiomyopathy; BSA = body surface area; EDV = end diastolic volume; EF = ejection fraction; ITF = International Task Force; LBBB = left bundle-branch block; LGE = late gadolinium<br \/>\nenhancement; LV = left ventricle; RBBB = right bundle-branch block; RV = right ventricle; RVOT = right ventricular outflow tract; CE-CMR = contrast enhanced-cardiovascular magnetic resonance; EMB = endomyocardial biopsy<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Mattesi G, Cipriani A, Bauce B, Rigato I, Zorzi A, Corrado D. Arrhythmogenic Left Ventricular Cardiomyopathy: Genotype-Phenotype Correlations and New Diagnostic Criteria. J Clin Med. 2021 May 20;10(10):2212. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/34065276\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<li>Corrado D, Zorzi A, Cipriani A, Bauce B, Bariani R, Beffagna G, De Lazzari M, Migliore F, Pilichou K, Rampazzo A, Rigato I, Rizzo S, Thiene G, Perazzolo Marra M, Basso C. Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy. J Am Heart Assoc. 2021 Sep 21;10(18):e021987. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/34533054\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Nov 26, 2021.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[26],"tags":[118,120,125,16,15,14,13,119,292,291],"class_list":["post-9552","post","type-post","status-publish","format-standard","hentry","category-cardiology","tag-arrhythmogenic","tag-arritmogenica","tag-cardiomyopathy","tag-criteria","tag-criterios","tag-diagnostic","tag-diagnostico","tag-miocardiopatia","tag-new","tag-nuevos"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9552","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=9552"}],"version-history":[{"count":15,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9552\/revisions"}],"predecessor-version":[{"id":9575,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9552\/revisions\/9575"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=9552"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=9552"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=9552"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}