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Myocarditic Appearance of Arrhythmogenic Right Ventricular Cardiomyopathy

2010; Lippincott Williams & Wilkins; Volume: 122; Issue: 21 Linguagem: Inglês

10.1161/circulationaha.110.960096

1524-4539

Andrew N. Jordan, Jonathan Lyne, Ranil de Silva, Tom Wong,

Viral Infections and Immunology Research

HomeCirculationVol. 122, No. 21Myocarditic Appearance of Arrhythmogenic Right Ventricular Cardiomyopathy Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessBrief ReportPDF/EPUBMyocarditic Appearance of Arrhythmogenic Right Ventricular Cardiomyopathy Andrew N. Jordan, MRCP, Jonathan Lyne, MRCP, Ranil De Silva, PhD and Tom Wong, MD Andrew N. JordanAndrew N. Jordan From the Royal Brompton Hospital, London, United Kingdom. , Jonathan LyneJonathan Lyne From the Royal Brompton Hospital, London, United Kingdom. , Ranil De SilvaRanil De Silva From the Royal Brompton Hospital, London, United Kingdom. and Tom WongTom Wong From the Royal Brompton Hospital, London, United Kingdom. Originally published23 Nov 2010https://doi.org/10.1161/CIRCULATIONAHA.110.960096Circulation. 2010;122:e556–e557A 48-year-old man presented with syncope due to tachycardia of left ventricular origin and was electrically cardioverted to sinus rhythm. There was no medical history of cardiac disease or family history of sudden cardiac death. Resting electrocardiography displayed epsilon waves (Figure, A). Coronary angiography revealed unobstructed arteries. Transthoracic echocardiography (Movies I and II) showed normal left ventricular dimensions with mild systolic dysfunction. The right ventricle was mildly dilated, with reduced systolic function but no regional wall-motion abnormalities or aneurysms. The right ventricular outflow tract measured 41 mm.Download figureDownload PowerPointFigure. A, Epsilon waves. B, Short-axis image at midventricular level. Late enhancement of the right ventricular border of the septum is seen, extending into epicardium of the anterior and inferior walls. Further late enhancement is seen throughout the right ventricular epicardium. C, Two-chamber view of the left ventricle. Extensive patchy enhancement was seen throughout the left ventricular mid wall and epicardium. D-E, Four-chamber and midventricular short-axis short TI inversion recovery sequences demonstrating fat suppression in the right ventricular myocardium.Cardiac magnetic resonance (CMR) imaging demonstrated a dilated right ventricle and biventricular systolic dysfunction (Table; Movies III and IV). Short-axis imaging at the midventricular level after injection of gadolinium at the midventricular level (Figure, B) identified patchy late enhancement of the right ventricular border of the septum, which extended into the subepicardium of the anterior and inferior walls. A 2-chamber view of the left ventricle (Figure, C) demonstrated extensive midmyocardial and subepicardial enhancement. In addition, short TI inversion recovery sequences (Figure, D and E) revealed fatty infiltration of the right ventricle in the absence of myocardial edema. The CMR differential diagnosis included chronic myocarditis and arrhythmogenic right ventricular cardiomyopathy (ARVC). Further investigation confirmed a mutation in the desmoplakin gene, which supports a diagnosis of this variant of ARVC.Table. Ventricular Wall Dimensions From CMR ImagingEDV, mLESV, mLSV, mLEF, %Mass Index, g/m2LV15883754960RV195 (88–227)118 (23–103)77 (52–138)39 (47–74)…EDV indicates end-diastolic volume; ESV, end-systolic volume; SV, stroke volume; EF, ejection fraction; LV, left ventricle; and RV, right ventricle.Normal ranges for right ventricular volumes are given in parentheses.The features presented fulfill the recently modified Task Force Criteria for the diagnosis of ARVC, produced with the aim of increasing the sensitivity of ARVC diagnosis.1 However, diagnostic doubt was raised because the CMR findings shown are typical of chronic myocarditis,2 with severe biventricular impairment and extensive diffuse midmyocardial and subepicardial late gadolinium enhancement, particularly of the left ventricle. Of note, CMR findings of late gadolinium enhancement are not included in the recently modified diagnostic criteria for ARVC, perhaps reflecting the difficulty in reliably determining pathological right ventricular enhancement patterns and underrepresentation of the left ventricular dominant subtype in clinical case series.An explanation for these CMR appearances is offered by the suggestion that diffuse myocardial inflammation is part of the ARVC disease process. An association between myocarditis and ARVC has been postulated previously, supported by the frequent demonstration of myocardial inflammation in postmortem histological analysis3 together with the presence of cardiotropic viruses in diseased hearts.4 However, the precise link between the 2 conditions has yet to be determined; these findings may indicate an inflammatory process related to progression of the disease itself or an increased susceptibility to myocardial infection due to apoptosis and cell debris within the diseased myocardium.In the present case, the demonstration of CMR features typical of chronic myocarditis in a patient with ARVC confirms the close relationship between these 2 conditions, with extensive involvement of the left ventricle characteristic of the desmoplakin variant of ARVC. Because images were taken during a period of intermittent arrhythmia, the CMR appearance may illustrate an "active myocarditic" phase of the ARVC disease process.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/122/21/e556/DC1.Correspondence to Andrew N. Jordan, MRCP, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, United Kingdom. E-mail [email protected]org.ukReferences1. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Protonotarios N, Saffitz JE, Sanborn DM, Steinberg JS, Tandri H, Thiene G, Towbin JA, Tsatsopoulou A, Wihter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Circulation. 2010; 121:1533–1541.LinkGoogle Scholar2. Friedrich MG, Sechtem U, Schulz-Menger J, Holmvang G, Alakija P, Cooper LT, White JA, Abdel-Aty H, Gutberlet M, Prasad S, Aletras A, Laissy JP, Paterson I, Filipchuk NG, Kumar A, Pauschinger M, Liu P; International Consensus Group on Cardiovascular Magnetic Resonance in Myocarditis. Cardiovascular magnetic resonance in myocarditis: a JACC White Paper. J Am Coll Cardiol. 2009; 53:1475–1487.CrossrefMedlineGoogle Scholar3. Burke AP, Farb A, Tashko G, Virmani R. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases?Circulation. 1998; 97:1571–1580.LinkGoogle Scholar4. Bowles NE, Ni J, Marcus F, Towbin JA. The detection of cardiotropic viruses in the myocardium of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2002; 39:892–895.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By McKenna W and Caforio A (2022) Myocardial Inflammation and Sudden Death in the Inherited Cardiomyopathies, Canadian Journal of Cardiology, 10.1016/j.cjca.2022.01.004, 38:4, (427-438), Online publication date: 1-Apr-2022. Asatryan B, Asimaki A, Landstrom A, Khanji M, Odening K, Cooper L, Marchlinski F, Gelzer A, Semsarian C, Reichlin T, Owens A and Chahal C (2021) Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review, Circulation, 144:20, (1646-1655), Online publication date: 16-Nov-2021. Ponsiglione A, Puglia M, Morisco C, Barbuto L, Rapacciuolo A, Santoro M, Spinelli L, Trimarco B, Cuocolo A and Imbriaco M (2016) A unique association of arrhythmogenic right ventricular dysplasia and acute myocarditis, as assessed by cardiac MRI: a case report, BMC Cardiovascular Disorders, 10.1186/s12872-016-0412-2, 16:1, Online publication date: 1-Dec-2016. Paylor B, Fernandes J, McManus B and Rossi F (2013) Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy, F1000Research, 10.12688/f1000research.2-141.v1, 2, (141) Tanawuttiwat T, Sager S, Hare J and Myerburg R (2013) Myocarditis and ARVC/D: Variants or mimics?, Heart Rhythm, 10.1016/j.hrthm.2013.06.008, 10:10, (1544-1548), Online publication date: 1-Oct-2013. Rubino M, Scatteia A, Frisso G, Pacileo G, Caiazza M, Pascale C, Guarini P, Limongelli G and Dellegrottaglie S (2021) Imaging the "Hot Phase" of a Familiar Left-Dominant Arrhythmogenic Cardiomyopathy, Genes, 10.3390/genes12121933, 12:12, (1933) November 23, 2010Vol 122, Issue 21 Advertisement Article InformationMetrics © 2010 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.110.960096PMID: 21098455 Originally publishedNovember 23, 2010 PDF download Advertisement SubjectsCardiomyopathyComputerized Tomography (CT)Electrocardiology (ECG)