Arrhythmogenic Right Ventricular Cardiomyopathy in a Pediatric Patient
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for developme...
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Published in | JACC. Case reports Vol. 2; no. 6; pp. 919 - 924 |
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Main Authors | , , , , , , , |
Format | Journal Article |
Language | English |
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01.06.2020
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Abstract | Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.)
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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically… |
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AbstractList | Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (
). Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.) Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.).Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.). Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. ( Level of Difficulty: Beginner. ) Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically… Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically confirmed ARVC who presented with syncope, ventricular arrhythmia, and biventricular myocardial dysfunction. This case highlights the need for development of pediatric ARVC diagnosis criteria specific for pediatric patients and discusses potential diagnostic improvement using echocardiographic deformation imaging. (Level of Difficulty: Beginner.) [Display omitted] Arrhythmogenic right ventricular cardiomyopathy (ARVC) is rarely diagnosed in childhood. We describe the case of a 9-year-old girl with genetically… |
Author | Marcelis, Carlo te Riele, Anneline S.J.M. Bosboom, Dennis Teske, Arco J. Velthuis, Birgitta K. Evertz, Reinder Udink ten Cate, Floris E.A. Roudijk, Rob W. |
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Keywords | ARVC desmosomal mutations genetic screening deformation imaging ventricular tachycardia arrhythmogenic cardiomyopathy pediatrics ECG arrhythmogenic right ventricular cardiomyopathy CMR DCM ARVC, arrhythmogenic right ventricular cardiomyopathy DCM, dilated cardiomyopathy ECG, electrocardiogram CMR, cardiac magnetic resonance |
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References | Marcus, Mavinkurve-Groothuis, Barends (bib9) 2011; 24 Marcus, McKenna, Sherrill (bib2) 2010; 121 Levy, Sanchez Mejia, Machefsky, Fowler, Holland, Singh (bib10) 2014; 27 Borgquist, Haugaa, Gilljam (bib7) 2014; 15 Towbin, McKenna, Abrams (bib3) 2019; 16 Maceira, Prasad, Khan, Pennell (bib6) 2006; 27 Mast, Teske, Walmsley (bib1) 2016; 68 Te Riele, James, Sawant (bib5) 2015; 1 DeWitt, Chandler, Hylind (bib4) 2019; 74 Bhonsale, Groeneweg, James (bib8) 2015; 36 Towbin (10.1016/j.jaccas.2020.01.006_bib3) 2019; 16 Maceira (10.1016/j.jaccas.2020.01.006_bib6) 2006; 27 Borgquist (10.1016/j.jaccas.2020.01.006_bib7) 2014; 15 Marcus (10.1016/j.jaccas.2020.01.006_bib9) 2011; 24 Mast (10.1016/j.jaccas.2020.01.006_bib1) 2016; 68 Levy (10.1016/j.jaccas.2020.01.006_bib10) 2014; 27 Marcus (10.1016/j.jaccas.2020.01.006_bib2) 2010; 121 DeWitt (10.1016/j.jaccas.2020.01.006_bib4) 2019; 74 Bhonsale (10.1016/j.jaccas.2020.01.006_bib8) 2015; 36 Te Riele (10.1016/j.jaccas.2020.01.006_bib5) 2015; 1 |
References_xml | – volume: 1 start-page: 551 year: 2015 end-page: 560 ident: bib5 article-title: Arrhythmogenic right ventricular dysplasia/cardiomyopathy in the pediatric population: clinical characterization and comparison with adult-onset disease publication-title: J Am Coll Cardiol EP contributor: fullname: Sawant – volume: 121 start-page: 1533 year: 2010 end-page: 1541 ident: bib2 article-title: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria publication-title: Circulation contributor: fullname: Sherrill – volume: 27 start-page: 2879 year: 2006 end-page: 2888 ident: bib6 article-title: Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance publication-title: Eur Heart J contributor: fullname: Pennell – volume: 27 start-page: 549 year: 2014 end-page: 560 ident: bib10 article-title: Normal ranges of right ventricular systolic and diastolic strain measures in children: a systematic review and meta-analysis publication-title: J Am Soc Echocardiogr contributor: fullname: Singh – volume: 68 start-page: 2185 year: 2016 end-page: 2197 ident: bib1 article-title: Right ventricular imaging and computer simulation for electromechanical substrate characterization in arrhythmogenic right ventricular cardiomyopathy publication-title: J Am Coll Cardiol contributor: fullname: Walmsley – volume: 36 start-page: 847 year: 2015 end-page: 855 ident: bib8 article-title: Impact of genotype on clinical course in arrhythmogenic right ventricular dysplasia/cardiomyopathy-associated mutation carriers publication-title: Eur Heart J contributor: fullname: James – volume: 24 start-page: 625 year: 2011 end-page: 636 ident: bib9 article-title: Reference values for myocardial two-dimensional strain echocardiography in a healthy pediatric and young adult cohort publication-title: J Am Soc Echocardiogr 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10.1016/j.jacc.2016.08.061 contributor: fullname: Mast – volume: 74 start-page: 346 year: 2019 ident: 10.1016/j.jaccas.2020.01.006_bib4 article-title: Phenotypic manifestations of arrhythmogenic cardiomyopathy in children and adolescents publication-title: J Am Coll Cardiol doi: 10.1016/j.jacc.2019.05.022 contributor: fullname: DeWitt – volume: 16 start-page: e373 year: 2019 ident: 10.1016/j.jaccas.2020.01.006_bib3 article-title: 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy publication-title: Heart Rhythm doi: 10.1016/j.hrthm.2019.09.019 contributor: fullname: Towbin – volume: 24 start-page: 625 year: 2011 ident: 10.1016/j.jaccas.2020.01.006_bib9 article-title: Reference values for myocardial two-dimensional strain echocardiography in a healthy pediatric and young adult cohort publication-title: J Am Soc Echocardiogr doi: 10.1016/j.echo.2011.01.021 contributor: fullname: Marcus |
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SubjectTerms | arrhythmogenic cardiomyopathy arrhythmogenic right ventricular cardiomyopathy deformation imaging desmosomal mutations genetic screening Mini-Focus Issue: Cardiomyopathies pediatrics ventricular tachycardia |
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Title | Arrhythmogenic Right Ventricular Cardiomyopathy in a Pediatric Patient |
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