FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2106133054> ?p ?o ?g. }

• W2106133054 abstract "HomeCirculationVol. 104, No. 23“Petrified” Right Ventricle in Long-Standing Naxos Arrhythmogenic Right Ventricular Cardiomyopathy Free AccessOtherPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessOtherPDF/EPUB“Petrified” Right Ventricle in Long-Standing Naxos Arrhythmogenic Right Ventricular Cardiomyopathy Cristina Basso, Adalena Tsatsopoulou, Gaetano Thiene, Aris Anastasakis, Marialuisa Valente and Nikos Protonotarios Cristina BassoCristina Basso From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author , Adalena TsatsopoulouAdalena Tsatsopoulou From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author , Gaetano ThieneGaetano Thiene From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author , Aris AnastasakisAris Anastasakis From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author , Marialuisa ValenteMarialuisa Valente From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author and Nikos ProtonotariosNikos Protonotarios From the Institute of Pathology, University of Padua, Italy (C.B., G.T., M.V.); Yannis Protonotarios Medical Center Hora Naxos, Greece (A.T., N.P.); and the Department of Cardiology, University of Athens, Greece (A.A.). Search for more papers by this author Originally published4 Dec 2001https://doi.org/10.1161/hc4701.098570Circulation. 2001;104:e132–e133A 19-year-old asymptomatic man was evaluated because of his sister’s sudden death due to Naxos disease, a recessive form of arrhythmogenic right ventricular (RV) cardiomyopathy associated with palmoplantar keratoderma and woolly hair. It is caused by a plakoglobin mutation. Twelve-lead ECG showed inverted T waves in V1 through V6, incomplete right bundle branch block, and epsilon waves (Figure 1). Frequent ventricular extrasystoles were recorded on a 24-hour ECG. On echocardiography and angiography, the right ventricle was dilated and diffusely hypokinetic, but the left ventricle appeared normal. During 14 years of follow-up, he had 2 episodes of well-tolerated sustained ventricular tachycardia followed by biventricular progression leading to heart failure (New York Heart Association class III to IV) without arrhythmias. The patient died at 33 years of age while waiting for heart transplantation. Download figureDownload PowerPointFigure 1. The 12-lead ECG shows inverted T waves (V1 to V6), incomplete right bundle branch block (QRS duration, 140 ms in V1), and epsilon waves.At gross examination and nuclear magnetic resonance (Figures 2 and 3), huge cardiomegaly (heart weight, 630 g) and paper-thin RV walls with transmural myocardial loss were evident, together with subepicardial left ventricular fatty infiltration. A calcified fibrous plaque, 7×9 cm in size, was discovered at the level of the RV outflow tract and confirmed by postmortem x-ray (Figure 4). Histological study showed transmural right and left ventricular myocardial atrophy, with fibrofatty replacement embedding a few surviving myocytes. The peculiar massive calcification of the RV outflow tract, possibly functioning as a ventricular disconnection, might explain the decreased arrhythmogenicity at the end-stage. Download figureDownload PowerPointFigure 2. Parasternal long-axis view of the heart shows paper-thin RV walls, transmural myocardial loss, subepicardial left ventricular fatty infiltration, and calcified fibrous plaque at the level of RV outflow tract.Download figureDownload PowerPointFigure 3. Corresponding long-axis nuclear magnetic resonance scan with diffuse bright signal on anterior RV wall and subepicardial bright signal on the left ventricular wall.Download figureDownload PowerPointFigure 4. Postmortem x-ray reveals diffuse calcification of the anterior RV wall.The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke‘s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke‘s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.Supported by the 5th Framework Program Research and Technological Development of the European Commission, Contract N. QLG1-CT-2000-01091 “ARVC/D.”FootnotesCorrespondence to Gaetano Thiene, MD, FESC, Istituto di Anatomia Patologica, Via A. Gabelli 61, 35121 Padova, Italy. E-mail [email protected] eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetailsCited By Lodato V, Parlapiano G, Calì F, Silvetti M, Adorisio R, Armando M, El Hachem M, Romanzo A, Dionisi-Vici C, Digilio M, Novelli A, Drago F, Raponi M and Baban A (2022) Cardiomyopathies in Children and Systemic Disorders When Is It Useful to Look beyond the Heart?, Journal of Cardiovascular Development and Disease, 10.3390/jcdd9020047, 9:2, (47) Protonotarios I, Asimaki A, Xylouri Z, Protonotarios A and Tsatsopoulou A (2022) Clinical and Molecular Aspects of Naxos Disease, Heart Failure Clinics, 10.1016/j.hfc.2021.07.010, 18:1, (89-99), Online publication date: 1-Jan-2022. Leopoulou M, Mattsson G, Kåks I and Magnusson P (2021) Naxos Disease: Current Knowledge and Future Advances Cardiomyopathy - Disease of the Heart Muscle, 10.5772/intechopen.96020 Leopoulou M, Mattsson G, LeQuang J, Pergolizzi J, Varrassi G, Wallhagen M and Magnusson P (2020) Naxos disease – a narrative review, Expert Review of Cardiovascular Therapy, 10.1080/14779072.2020.1828064, 18:11, (801-808), Online publication date: 1-Nov-2020. Tsatsopoulou A and Bossone E (2018) Common presentation of rare diseases: Arrhythmogenic right ventricular cardiomyopathy and its mimics, International Journal of Cardiology, 10.1016/j.ijcard.2018.01.021, 257, (371-377), Online publication date: 1-Apr-2018. Sarquella-Brugada G, Campuzano O, Berruezo A and Brugada J (2014) Ventricular Tachycardiac and Sudden Arrhythmic Death Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care, 10.1007/978-1-4471-4619-3_138, (2971-2998), . Protonotarios N and Tsatsopoulou A (2011) Arrhythmogenic Cardiomyopathy: Clinical Presentation, Cardiac Electrophysiology Clinics, 10.1016/j.ccep.2011.02.013, 3:2, (227-235), Online publication date: 1-Jun-2011. Pilichou K, Remme C, Basso C, Campian M, Rizzo S, Barnett P, Scicluna B, Bauce B, van den Hoff M, de Bakker J, Tan H, Valente M, Nava A, Wilde A, Moorman A, Thiene G and Bezzina C (2009) Myocyte necrosis underlies progressive myocardial dystrophy in mouse dsg2-related arrhythmogenic right ventricular cardiomyopathy, Journal of Experimental Medicine, 10.1084/jem.20090641, 206:8, (1787-1802), Online publication date: 3-Aug-2009. Bolling M and Jonkman M (2009) Skin and heart: une liaison dangereuse, Experimental Dermatology, 10.1111/j.1600-0625.2009.00908.x, 18:8, (658-668), Online publication date: 1-Aug-2009. Basso C and Thiene G Autopsy and Endomyocardial Biopsy Findings Arrhythmogenic RV Cardiomyopathy/Dysplasia, 10.1007/978-88-470-0490-0_5, (29-44) Protonotarios N and Tsatsopoulou A Advances in Genetics: Recessive Forms Arrhythmogenic RV Cardiomyopathy/Dysplasia, 10.1007/978-88-470-0490-0_3, (15-20) Protonotarios N and Tsatsopoulou A (2004) Naxos disease and Carvajal syndrome, Cardiovascular Pathology, 10.1016/j.carpath.2004.03.609, 13:4, (185-194), Online publication date: 1-Jul-2004. Kaplan S, Gard J, Protonotarios N, Tsatsopoulou A, Spiliopoulou C, Anastasakis A, Squarcioni C, McKenna W, Thiene G, Basso C, Brousse N, Fontaine G and Saffitz J (2004) Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease), Heart Rhythm, 10.1016/j.hrthm.2004.01.001, 1:1, (3-11), Online publication date: 1-May-2004. December 4, 2001Vol 104, Issue 23 Advertisement Article InformationMetrics https://doi.org/10.1161/hc4701.098570 Originally publishedDecember 4, 2001 PDF download Advertisement" @default.
• W2106133054 created "2016-06-24" @default.
• W2106133054 creator A5022167195 @default.
• W2106133054 creator A5029485255 @default.
• W2106133054 creator A5055738866 @default.
• W2106133054 creator A5074347698 @default.
• W2106133054 creator A5077749268 @default.
• W2106133054 creator A5085386690 @default.
• W2106133054 date "2001-12-04" @default.
• W2106133054 modified "2023-10-16" @default.
• W2106133054 title "“Petrified” Right Ventricle in Long-Standing Naxos Arrhythmogenic Right Ventricular Cardiomyopathy" @default.
• W2106133054 doi "https://doi.org/10.1161/hc4701.098570" @default.
• W2106133054 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/11733410" @default.
• W2106133054 hasPublicationYear "2001" @default.
• W2106133054 type Work @default.
• W2106133054 sameAs 2106133054 @default.
• W2106133054 citedByCount "19" @default.
• W2106133054 countsByYear W21061330542012 @default.
• W2106133054 countsByYear W21061330542013 @default.
• W2106133054 countsByYear W21061330542018 @default.
• W2106133054 countsByYear W21061330542020 @default.
• W2106133054 countsByYear W21061330542021 @default.
• W2106133054 countsByYear W21061330542022 @default.
• W2106133054 crossrefType "journal-article" @default.
• W2106133054 hasAuthorship W2106133054A5022167195 @default.
• W2106133054 hasAuthorship W2106133054A5029485255 @default.
• W2106133054 hasAuthorship W2106133054A5055738866 @default.
• W2106133054 hasAuthorship W2106133054A5074347698 @default.
• W2106133054 hasAuthorship W2106133054A5077749268 @default.
• W2106133054 hasAuthorship W2106133054A5085386690 @default.
• W2106133054 hasBestOaLocation W21061330541 @default.
• W2106133054 hasConcept C126322002 @default.
• W2106133054 hasConcept C164705383 @default.
• W2106133054 hasConcept C2777892804 @default.
• W2106133054 hasConcept C2778198053 @default.
• W2106133054 hasConcept C2778797674 @default.
• W2106133054 hasConcept C2778921608 @default.
• W2106133054 hasConcept C71924100 @default.
• W2106133054 hasConceptScore W2106133054C126322002 @default.
• W2106133054 hasConceptScore W2106133054C164705383 @default.
• W2106133054 hasConceptScore W2106133054C2777892804 @default.
• W2106133054 hasConceptScore W2106133054C2778198053 @default.
• W2106133054 hasConceptScore W2106133054C2778797674 @default.
• W2106133054 hasConceptScore W2106133054C2778921608 @default.
• W2106133054 hasConceptScore W2106133054C71924100 @default.
• W2106133054 hasIssue "23" @default.
• W2106133054 hasLocation W21061330541 @default.
• W2106133054 hasLocation W21061330542 @default.
• W2106133054 hasOpenAccess W2106133054 @default.
• W2106133054 hasPrimaryLocation W21061330541 @default.
• W2106133054 hasRelatedWork W2000547619 @default.
• W2106133054 hasRelatedWork W2021752810 @default.
• W2106133054 hasRelatedWork W2087414938 @default.
• W2106133054 hasRelatedWork W2184433909 @default.
• W2106133054 hasRelatedWork W2404087378 @default.
• W2106133054 hasRelatedWork W2728772236 @default.
• W2106133054 hasRelatedWork W2795420160 @default.
• W2106133054 hasRelatedWork W4226001377 @default.
• W2106133054 hasRelatedWork W71673504 @default.
• W2106133054 hasRelatedWork W2186107776 @default.
• W2106133054 hasVolume "104" @default.
• W2106133054 isParatext "false" @default.
• W2106133054 isRetracted "false" @default.
• W2106133054 magId "2106133054" @default.
• W2106133054 workType "article" @default.