arrhythmogenic right ventricular dysplasia

Summary

Summary: A congenital cardiomyopathy in which transmural infiltration of adipose tissue results in weakness and aneurysmal bulging of the infundibulum, apex, and posterior basilar region of the right ventricle and leads to ventricular tachycardia arising in the right ventricle. (Dorland, 28th ed)

Top Publications

  1. ncbi Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria
    Frank I Marcus
    University of Arizona, Tucson, Ariz, USA
    Circulation 121:1533-41. 2010
  2. ncbi The p.A897KfsX4 frameshift variation in desmocollin-2 is not a causative mutation in arrhythmogenic right ventricular cardiomyopathy
    Marzia De Bortoli
    Department of Biology, University of Padua, Padua, Italy
    Eur J Hum Genet 18:776-82. 2010
  3. ncbi Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice
    Veronique Fressart
    AP HP, Hopital Pitie Salpetriere, Service de Biochimie, Unité de Cardiogénétique et Myogénétique, Paris, France
    Europace 12:861-8. 2010
  4. ncbi Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene
    Nancy D Merner
    Faculty of Medicine, Memorial University, St John s, Newfoundland and Labrador A1B 3V6, Canada
    Am J Hum Genet 82:809-21. 2008
  5. ncbi Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Mark M Awad
    Johns Hopkins University School of Medicine and Johns Hopkins University Cellular and Molecular Medicine Program, Baltimore, MD, USA
    Nat Clin Pract Cardiovasc Med 5:258-67. 2008
  6. ncbi Arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Gaetano Thiene
    Pathological Anatomy, Department of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy
    Orphanet J Rare Dis 2:45. 2007
  7. ncbi Genetic fate mapping identifies second heart field progenitor cells as a source of adipocytes in arrhythmogenic right ventricular cardiomyopathy
    Raffaella Lombardi
    Center for Cardiovascular Genetics, Brown Foundation Institute of Molecular Medicine, The University of Texas Health Sciences Center, 6770 Bertner Street, Houston, TX 77030, USA
    Circ Res 104:1076-84. 2009
  8. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnostic task force criteria: impact of new task force criteria
    Moniek G P J Cox
    Department of Cardiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands
    Circ Arrhythm Electrophysiol 3:126-33. 2010
  9. ncbi Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria
    Frank I Marcus
    University of Arizona, Tucson, AZ, USA
    Eur Heart J 31:806-14. 2010
  10. ncbi Arrhythmogenic right ventricular dysplasia: a United States experience
    Darshan Dalal
    Division of Cardiology, The Johns Hopins University School of Medicine, Baltimore, MD, USA
    Circulation 112:3823-32. 2005

Research Grants

  1. Multidisciplinary Study of Right Ventricular Dysplasia
    Wojciech Zareba; Fiscal Year: 2005
  2. Maturation of Normal and Disease-specific Human Stem Cell-derived Cardiomyocytes
    Huei Sheng Vincent Chen; Fiscal Year: 2010
  3. A Clinical & Molecular Analysis of the Brugada Syndrome
    Barry London; Fiscal Year: 2003
  4. The molecular mechanisms underlying arrhythmogenic right ventricular dysplasia/ca
    Farah Sheikh; Fiscal Year: 2010
  5. The molecular mechanisms underlying arrhythmogenic right ventricular dysplasia/ca
    Farah Sheikh; Fiscal Year: 2010
  6. A Clinical & Molecular Analysis of the Brugada Syndrome
    Barry London; Fiscal Year: 2005
  7. Role of Desmosomal Adhesion in Carcinogenesis
    MY MAHONEY; Fiscal Year: 2009
  8. Role of Desmosomal Adhesion in Carcinogenesis
    MY GEORGIA MAHONEY; Fiscal Year: 2011
  9. Genetic Investigation of Arrhythmogenic Right Ventricular Dysplasia
    Daniel Judge; Fiscal Year: 2007
  10. CLONING OF A POTENTIAL REGULATOR OF THE DHAND FACTOR
    Mark Russell; Fiscal Year: 2002

Detail Information

Publications181 found, 100 shown here

  1. ncbi Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria
    Frank I Marcus
    University of Arizona, Tucson, Ariz, USA
    Circulation 121:1533-41. 2010
    ..Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease...
  2. ncbi The p.A897KfsX4 frameshift variation in desmocollin-2 is not a causative mutation in arrhythmogenic right ventricular cardiomyopathy
    Marzia De Bortoli
    Department of Biology, University of Padua, Padua, Italy
    Eur J Hum Genet 18:776-82. 2010
    ....
  3. ncbi Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice
    Veronique Fressart
    AP HP, Hopital Pitie Salpetriere, Service de Biochimie, Unité de Cardiogénétique et Myogénétique, Paris, France
    Europace 12:861-8. 2010
    Five desmosomal genes have been recently implicated in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) but the clinical impact of genetics remains poorly understood...
  4. ncbi Arrhythmogenic right ventricular cardiomyopathy type 5 is a fully penetrant, lethal arrhythmic disorder caused by a missense mutation in the TMEM43 gene
    Nancy D Merner
    Faculty of Medicine, Memorial University, St John s, Newfoundland and Labrador A1B 3V6, Canada
    Am J Hum Genet 82:809-21. 2008
    ....
  5. ncbi Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Mark M Awad
    Johns Hopkins University School of Medicine and Johns Hopkins University Cellular and Molecular Medicine Program, Baltimore, MD, USA
    Nat Clin Pract Cardiovasc Med 5:258-67. 2008
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy estimated to affect approximately 1 in 5,000 individuals...
  6. ncbi Arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Gaetano Thiene
    Pathological Anatomy, Department of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy
    Orphanet J Rare Dis 2:45. 2007
    ..Preparticipation screening for sport eligibility has been proven to be effective in detecting asymptomatic patients and sport disqualification has been life-saving, substantially declining sudden death in young athletes...
  7. ncbi Genetic fate mapping identifies second heart field progenitor cells as a source of adipocytes in arrhythmogenic right ventricular cardiomyopathy
    Raffaella Lombardi
    Center for Cardiovascular Genetics, Brown Foundation Institute of Molecular Medicine, The University of Texas Health Sciences Center, 6770 Bertner Street, Houston, TX 77030, USA
    Circ Res 104:1076-84. 2009
    ..We conclude adipocytes in arrhythmogenic right ventricular cardiomyopathy originate from the second heart field cardiac progenitors, which switch to an adipogenic fate because of suppressed canonical Wnt signaling by nuclear plakoglobin...
  8. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnostic task force criteria: impact of new task force criteria
    Moniek G P J Cox
    Department of Cardiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands
    Circ Arrhythm Electrophysiol 3:126-33. 2010
    b>Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) Diagnostic Task Force Criteria (TFC) proposed in 1994 are highly specific but lack sensitivity...
  9. ncbi Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria
    Frank I Marcus
    University of Arizona, Tucson, AZ, USA
    Eur Heart J 31:806-14. 2010
    ..Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease...
  10. ncbi Arrhythmogenic right ventricular dysplasia: a United States experience
    Darshan Dalal
    Division of Cardiology, The Johns Hopins University School of Medicine, Baltimore, MD, USA
    Circulation 112:3823-32. 2005
    BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by right ventricular dysfunction and ventricular arrhythmias...
  11. ncbi Mechanistic insights into arrhythmogenic right ventricular cardiomyopathy caused by desmocollin-2 mutations
    Katja Gehmlich
    Institute of Cardiovascular Science, University College London, London, UK
    Cardiovasc Res 90:77-87. 2011
    ..However, the underlying molecular processes are poorly understood...
  12. ncbi Predictors of appropriate implantable defibrillator therapies in patients with arrhythmogenic right ventricular dysplasia
    Jonathan P Piccini
    Division of Cardiology, Department of Medicine, The Johns Hopkins Hospital, 600 N. Wolfe Street, Carnegie 592, Baltimore, MD 21287, USA
    Heart Rhythm 2:1188-94. 2005
    BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by ventricular arrhythmias and sudden cardiac death...
  13. ncbi Abnormal connexin43 in arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 mutations
    Lee M Fidler
    Heart Centre Cardiology Division, The Hospital for Sick Children, Toronto, ON, Canada
    J Cell Mol Med 13:4219-28. 2009
    ..Reduced connexin43 expression and localization to the intercalated disk occurs in heterozygous human PKP-2 mutations, potentially explaining the delayed conduction and propensity to develop arrhythmias seen in this disease...
  14. ncbi Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice
    Paulus Kirchhof
    Department of Cardiology and Angiology, Hospital of the University of Muenster, Germany
    Circulation 114:1799-806. 2006
    ..To test the hypothesis that reduced desmosomal protein expression causes ARVC, we studied the cardiac effects of heterozygous plakoglobin deficiency in mice...
  15. ncbi Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy
    Brenda Gerull
    Max Delbrueck Center for Molecular Medicine, D 13092 Berlin Buch, Germany
    Nat Genet 36:1162-4. 2004
    ..In two kindreds with ARVC, disease was incompletely penetrant in most carriers of PKP2 mutations...
  16. ncbi Electrocardiographic features of arrhythmogenic right ventricular dysplasia/cardiomyopathy according to disease severity: a need to broaden diagnostic criteria
    Khurram Nasir
    Department of Medicine, The Johns Hopkins University, Baltimore, MD, USA
    Circulation 110:1527-34. 2004
    ..This feature also best distinguished ARVD/C (diffuse and localized) from RVOT. CONCLUSIONS: A prolonged S-wave upstroke in V1 through V3 is the most frequent ECG finding in ARVD/C and should be considered as a diagnostic ECG marker...
  17. ncbi Shared desmosome gene findings in early and late onset arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Boon Yew Tan
    Department of Medicine Cardiology, Johns Hopkins University School of Medicine, Rutland Avenue, Baltimore, MD 21205, USA
    J Cardiovasc Transl Res 3:663-73. 2010
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited form of cardiomyopathy with low penetrance and variable expressivity...
  18. ncbi Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management
    Srijita Sen-Chowdhry
    Department of Cardiology, The Heart Hospital, London, UK
    Am J Med 117:685-95. 2004
    ..Greater awareness of right ventricular dysplasia among physicians and judicious use of implantable cardioverter-defibrillators may help to prevent unnecessary deaths...
  19. ncbi Long-term efficacy of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Darshan Dalal
    Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    J Am Coll Cardiol 50:432-40. 2007
    ..the outcomes of radiofrequency catheter ablation (RFA) of ventricular tachycardia (VT) in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) patients...
  20. ncbi Assessment of inflammation in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Maria E Campian
    University of Amsterdam, Meibergdreef 9, Amsterdam, The Netherlands
    Eur J Nucl Med Mol Imaging 37:2079-85. 2010
    ..We aimed to establish whether cardiac inflammation can be assessed non-invasively in ARVC/D patients...
  21. ncbi Quantitative assessment of endomyocardial biopsy in arrhythmogenic right ventricular cardiomyopathy/dysplasia: an in vitro validation of diagnostic criteria
    Cristina Basso
    Pathological Anatomy, Department of Medico Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy
    Eur Heart J 29:2760-71. 2008
    ..The Task Force criteria for the in vivo diagnosis of ARVC/D include tissue characterization by EMB as a major criterion...
  22. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy: screening, diagnosis, and treatment
    Philippine Kies
    Department of Cardiology, Leiden University Medical Centre, Leiden, The Netherlands
    Heart Rhythm 3:225-34. 2006
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disorder characterized pathologically by fatty or fibrofatty replacement and electrical instability of the right ventricular myocardium...
  23. ncbi Epicardial ventricular tachycardia ablation a multicenter safety study
    Frederic Sacher
    Universite Bordeaux II, Hopital Cardiologique du Haut Leveque, Bordeaux Pessac, France
    J Am Coll Cardiol 55:2366-72. 2010
    ..The aim of this study was to perform a systematic evaluation of safety and midterm complications after epicardial ventricular tachycardia (VT) ablation...
  24. ncbi Novel missense mutations in exon 15 of desmoglein-2: role of the intracellular cadherin segment in arrhythmogenic right ventricular cardiomyopathy?
    Katja Gehmlich
    Institute of Cardiovascular Science, University College London, United Kingdom
    Heart Rhythm 7:1446-53. 2010
    ..Disease-causing mutations in desmosomal genes have been identified. A novel diagnostic feature, loss of immunoreactivity for plakoglobin from the intercalated disks, recently was proposed...
  25. ncbi Radiofrequency ablation of the ventricular tachycardia with arrhythmogenic right ventricular cardiomyopathy using non-contact mapping
    Yan Yao
    Clinical EP Lab and Cardiac Arrhythmia Service Center, Cardiovascular Institute, Fuwai Heart Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100037, China
    Pacing Clin Electrophysiol 30:526-33. 2007
    ..Intracardiac non-contact mapping provides a rapid and accurate isopotential mapping that facilitates catheter ablation of the ventricular tachyarrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC)...
  26. ncbi Prolonged RV endocardial activation duration: a novel marker of arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Harikrishna Tandri
    Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Heart Rhythm 6:769-75. 2009
    Parietal block, defined as intra right ventricular (RV) conduction slowing, is a major diagnostic criterion for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).
  27. ncbi Electroanatomic mapping of endocardial right ventricular activation as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia
    Christopher Reithmann
    Medizinische Klinik I, Klinikum Grosshadern, , , Germany
    Pacing Clin Electrophysiol 26:1308-16. 2003
    b>Arrhythmogenic right ventricular dysplasia is a structural heart disease characterized by fibrofatty degeneration of right ventricular myocardium and arrhythmias of right ventricular origin...
  28. ncbi A recessive mutation in desmoplakin causes arrhythmogenic right ventricular dysplasia, skin disorder, and woolly hair
    Ronny Alcalai
    Department of Medicine Mount Scopus, Jerusalem, Israel
    J Am Coll Cardiol 42:319-27. 2003
    ..The goal of this study was to analyze the genetic disorder of a family with cardiomyopathy, skin disorder, and woolly hair...
  29. ncbi Role of genetic testing in arrhythmogenic right ventricular cardiomyopathy/dysplasia
    C Barahona-Dussault
    Institut de Cardiologie de Montreal, Montreal, Quebec, Canada
    Clin Genet 77:37-48. 2010
    ....
  30. ncbi New ECG criteria in arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Moniek G P J Cox
    Department of Cardiology, University Medical Center Utrecht, 3584 CX Utrecht, The Netherlands
    Circ Arrhythm Electrophysiol 2:524-30. 2009
    ..changes, electric uncoupling, and surviving myocardial bundles in fibrofatty tissue characterize arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)...
  31. ncbi The cardiac desmosome and arrhythmogenic cardiomyopathies: from gene to disease
    Mario Delmar
    Division of Cardiovascular Medicine, University of Michigan Medical School, Ann Arbor, Mich, USA
    Circ Res 107:700-14. 2010
    ....
  32. ncbi Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy
    Eduardo Garcia-Gras
    Section of Cardiology and Center for Cardiac Development, Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    J Clin Invest 116:2012-21. 2006
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is a genetic disease caused by mutations in desmosomal proteins...
  33. ncbi Short- and long-term success of substrate-based mapping and ablation of ventricular tachycardia in arrhythmogenic right ventricular dysplasia
    Atul Verma
    Section of Pacing and Electrophysiology, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Circulation 111:3209-16. 2005
    ..instability, or noninducibility may limit ventricular tachycardia (VT) ablation in patients with arrhythmogenic right ventricular dysplasia (ARVD). Substrate-based mapping and ablation may overcome these limitations...
  34. ncbi Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy
    A Dénise den Haan
    Department of Medicine Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Circ Cardiovasc Genet 2:428-35. 2009
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited disorder typically caused by mutations in components of the cardiac desmosome...
  35. ncbi Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression
    Srijita Sen Chowdhry
    The Heart Hospital, University College London, United Kingdom
    Circulation 115:1710-20. 2007
    According to clinical-pathological correlation studies, the natural history of arrhythmogenic right ventricular dysplasia/cardiomyopathy is purported to progress from localized to global right ventricular dysfunction, followed by left ..
  36. ncbi Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia
    Harikrishna Tandri
    Division of Cardiology, The Johns Hopkins University, Baltimore, Maryland 21287, USA
    J Magn Reson Imaging 19:848-58. 2004
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is a familial cardiomyopathy that causes fibro-fatty replacement of the right ventricle (RV), leading to sudden death due to ventricular arrhythmias...
  37. ncbi Desmoplakin disease in arrhythmogenic right ventricular cardiomyopathy: early genotype-phenotype studies
    Srijita Sen-Chowdhry
    Eur Heart J 26:1582-4. 2005
  38. ncbi Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype-phenotype characterization of familial disease
    Petros Syrris
    Department of Medicine, The Heart Hospital, University College London and University College London Hospitals Trust, 16 18 Westmoreland Street, London W1G 8PH, UK
    Eur Heart J 28:581-8. 2007
    ..In this study, we aimed to clinically characterize probands and family members carrying a DSG2 mutation...
  39. ncbi MR Imaging of arrhythmogenic right ventricular cardiomyopathy: morphologic findings and interobserver reliability
    David A Bluemke
    Department of Radiology, Johns Hopkins Hospital, Baltimore, MD 21287, USA
    Cardiology 99:153-62. 2003
    ..However, the reliability of various MR imaging features for diagnosing ARVC/D is unknown. The purpose of this study was to determine which morphologic MR imaging features have the greatest interobserver reliability for diagnosing ARVC/D...
  40. ncbi Regional differences in systolic and diastolic function in arrhythmogenic right ventricular dysplasia/cardiomyopathy using magnetic resonance imaging
    Chandra Bomma
    Division of Cardiology (Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    Am J Cardiol 95:1507-11. 2005
    Global and regional biventricular functions were analyzed in 14 patients diagnosed with arrhythmogenic right ventricular dysplasia/cardiomyopathy using cine magnetic resonance imaging and compared with similar data from 18 age-matched ..
  41. ncbi Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: an electron microscopy investigation on endomyocardial biopsies
    Cristina Basso
    Institute of Pathological Anatomy, University of Padua, Italy
    Eur Heart J 27:1847-54. 2006
    ..CONCLUSION: The ultrastructural evidence of intercalated discs remodelling in ARVC, together with the positive screening of D protein encoding genes in half of probands, are in keeping with an intercellular junction cardiomyopathy...
  42. ncbi Arrhythmogenic right ventricular cardiomyopathy due to a novel plakophilin 2 mutation: wide spectrum of disease in mutation carriers within a family
    Prince J Kannankeril
    Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee 37232 9119, USA
    Heart Rhythm 3:939-44. 2006
    ..Mutations in plakophilin-2 (PKP2), a desmosomal protein, have been reported to underlie familial ARVC. We report a novel ARVC PKP2 mutation and present the clinical findings in three female mutation carriers...
  43. ncbi Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy - a scintigraphic and echocardiographic study
    Lena Lindstrom
    Department of Clinical Physiology, Faculty of Health Science, Linkoping University, Sweden
    Clin Physiol Funct Imaging 25:171-7. 2005
    ....
  44. ncbi Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a matter of fat?
    Cristina Basso
    Institute of Pathological Anatomy, University of Padua Medical School, Padua 35121, Italy
    Cardiovasc Pathol 14:37-41. 2005
    ....
  45. ncbi Regulatory mutations in transforming growth factor-beta3 gene cause arrhythmogenic right ventricular cardiomyopathy type 1
    Giorgia Beffagna
    Department of Biology, University of Padua, Via Ugo Bassi 58 B 35131 Padua, Italy
    Cardiovasc Res 65:366-73. 2005
    ..The objective of this study is to identify the gene involved in ARVD1, which has been elusive ever since its locus was mapped to chromosome 14q24.3...
  46. ncbi Advances in the diagnostic management of arrhythmogenic right ventricular dysplasia-cardiomyopathy
    Stefan Peters
    Klinikum Dorothea Christiane Erxleben gGmbH Quedlinburg, Department of Internal Medicine and Cardiology, Ditfurter Weg 24, 06484, Quedlinburg, Germany
    Int J Cardiol 113:4-11. 2006
    Latest advances in the diagnostic management of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) confirm that ARVD/C is not a rare disease (one affected in 1000-1250 inhabitants) and is of familial origin in 50-80% of ..
  47. ncbi Arrhythmogenic right ventricular cardiomyopathy/dysplasia: clinical impact of molecular genetic studies
    Domenico Corrado
    Circulation 113:1634-7. 2006
  48. ncbi Arrhythmogenic right ventricular dysplasia: ex vivo and in vivo fat detection with black-blood MR imaging
    Ernesto Castillo
    Russell H. Morgan Dept of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, MRI-143 Nelson Basement, Baltimore, MD 21287, USA
    Radiology 232:38-48. 2004
    ..imaging in the depiction of intramyocardial fat in cadaveric heart specimens and patients with arrhythmogenic right ventricular dysplasia (ARVD)...
  49. ncbi Results of ajmaline testing in patients with arrhythmogenic right ventricular dysplasia-cardiomyopathy
    Stefan Peters
    Klinikum Dorothea Christiane Erxleben gGmbH Quedlinburg, Department of Cardiology, Academic Teaching Hospital of the University Hospital Magdeburg, Ditfurter Weg 24, 06484 Quedlinburg, Germany
    Int J Cardiol 95:207-10. 2004
    An association between arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) and Brugada syndrome can be supposed according to several case reports...
  50. ncbi Prevalence of T-wave inversion beyond V1 in young normal individuals and usefulness for the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Frank I Marcus
    Am J Cardiol 95:1070-1. 2005
    ....
  51. ncbi Arrhythmogenic right ventricular dysplasia, a cell adhesion cardiomyopathy: insights into disease pathogenesis from preliminary genotype--phenotype assessment
    A A Tsatsopoulou
    Heart 92:1720-3. 2006
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is a genetically determined heart muscle disorder presenting clinically with even lethal ventricular arrhythmias, particularly in the young and athletes...
  52. ncbi Review on the genetics of arrhythmogenic right ventricular dysplasia
    Ewa Moric-Janiszewska
    Department of Biochemistry, Medical University of Silesia, Narcyzow 1, 41 200 Sosnowiec, Poland
    Europace 9:259-66. 2007
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is a clinical and pathologic entity whose diagnosis rests on electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and ..
  53. ncbi Marked lipomatous infiltration of the right ventricle: MRI findings in relation to arrhythmogenic right ventricular dysplasia
    Robson Macedo
    Russell H Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, MRI Bldg, Rm 143, 600 N Wolf St, Baltimore, MD 21287, USA
    AJR Am J Roentgenol 188:W423-7. 2007
    ..for second opinions about fatty infiltration of the right ventricular wall and suspected arrhythmogenic right ventricular dysplasia. CONCLUSION: Lipomatous infiltration with right ventricular thickness > or = 6 mm on MRI but ..
  54. ncbi Desmoglein-2 and desmocollin-2 mutations in dutch arrhythmogenic right ventricular dysplasia/cardiomypathy patients: results from a multicenter study
    Zahurul A Bhuiyan
    Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
    Circ Cardiovasc Genet 2:418-27. 2009
    ..major desmosomal genes, Plakophilin-2 (PKP2), Desmoglein-2 (DSG2), and Desmocollin-2 (DSC2), in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) patients...
  55. ncbi Epicardial substrate and outcome with epicardial ablation of ventricular tachycardia in arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Fermin C Garcia
    Cardiovascular Division, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, USA
    Circulation 120:366-75. 2009
    ..We sought to characterize the endocardial versus epicardial substrate, measure right ventricular free wall thickness, and determine epicardial ablation efficacy in patients with right ventricular cardiomyopathy/dysplasia...
  56. ncbi Morphologic variants of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy a genetics-magnetic resonance imaging correlation study
    Darshan Dalal
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    J Am Coll Cardiol 53:1289-99. 2009
    ..study was to determine the extent of left ventricular (LV) involvement in individuals predisposed to developing arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), and to investigate novel morphologic variants of ARVD/C.
  57. ncbi Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases?
    A P Burke
    Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC 20306 6000, USA
    Circulation 97:1571-80. 1998
    ..The relationship between arrhythmogenic right ventricular cardiomyopathy (ARVC) and pure fat replacement of the right ventricle is unclear...
  58. ncbi Arrhythmogenic right ventricular dysplasia
    Palaniappan Muthappan
    Division of Cardiology, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Prog Cardiovasc Dis 51:31-43. 2008
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV)...
  59. ncbi The locus of a novel gene responsible for arrhythmogenic right-ventricular dysplasia characterized by early onset and high penetrance maps to chromosome 10p12-p14
    D Li
    Department of Medicine, Section of Cardiology, Baylor College of Medicine, Houston, TX 77030, USA
    Am J Hum Genet 66:148-56. 2000
    ..Chromosomal localization of the ARVD gene is the first step in identification of the genetic defect and the unraveling of the molecular basis responsible for the pathogenesis of the disease...
  60. ncbi Characterization of HACD1 K64Q mutant found in arrhythmogenic right ventricular dysplasia patients
    Hiroyuki Konishi
    Laboratory of Biochemistry, Faculty of Pharmaceutical Sciences, Hokkaido University, Kita 12 jo, Nishi 6 choume, Kita ku, Sapporo 060 0812, Japan
    J Biochem 148:617-22. 2010
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is an autosomal dominant heart disease...
  61. ncbi Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)
    N Tiso
    Department of Biology, University of Padova, 35121 Padova, Italy
    Hum Mol Genet 10:189-94. 2001
    b>Arrhythmogenic right ventricular dysplasia type 2 (ARVD2, OMIM 600996) is an autosomal dominant cardiomyopathy, characterized by partial degeneration of the myocardium of the right ventricle, electrical instability and sudden death...
  62. ncbi Dispersion of ventricular depolarization-repolarization: a noninvasive marker for risk stratification in arrhythmogenic right ventricular cardiomyopathy
    P Turrini
    Department of Pathology, University of Padua Medical School, Padua, Italy
    Circulation 103:3075-80. 2001
    ..CONCLUSIONS: QRS dispersion (>/=40 ms) was the strongest independent predictor of sudden death in ARVC. Syncope, QT dispersion >65 ms, and negative T wave beyond V(1) refined arrhythmic risk stratification in these patients...
  63. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Hicham Z El Masry
    Krannert Institute of Cardiology, Indiana University, Room E375, 1801 N Senate Blvd, Suite E400, Indianapolis, IN 46202 1228, USA
    Expert Rev Cardiovasc Ther 6:249-60. 2008
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiac disease characterized by fibrofatty replacement of the cardiac myocytes...
  64. ncbi Arrhythmogenic right ventricular dysplasia presenting with severe right heart failure leading to abdominal ascites in a child
    Tevfik Karagoz
    Hacettepe University Faculty of Medicine, Department of Pediatrics, Section of Pediatric Cardiology, Ankara, Turkey
    Acta Cardiol 60:546-50. 2005
    This report describes a 12-year-old girl with arrhythmogenic right ventricular dysplasia, presenting with severe right ventricular failure symptoms including fatigue, abdominal pain and distension due to abdominal ascites.
  65. ncbi Arrhythmogenic right ventricular cardiomyopathy in the elderly: an uncommon finding using magnetic resonance imaging
    Anil-Martin Sinha
    Department of Cardiology, II. Medizinische Klinik, Klinikum Coburg, Ketschendorfer Str. 30, 96450 Coburg, Germany
    Europace 10:114-5. 2008
  66. ncbi Arrhythmogenic right ventricular cardiomyopathy in an 11-year-old girl and typical echocardiographic features
    Abdul Rahim Wong
    Pediatric Department, King Khalid University Hospital, Riyadh, Saudi Arabia
    Pediatr Cardiol 29:427-30. 2008
    ....
  67. ncbi Cardiac sarcoidosis imitating arrhythmogenic right ventricular dysplasia
    Kibar Yared
    Cardiac Ultrasound Laboratory, Massachusetts General Hospital, Boston, MA 02114-2696, USA
    Circulation 118:e113-5. 2008
  68. ncbi Economic evaluation of screening for familiar form of arrhythmogenic right ventricular cardiomyopathy in Poland
    Ewa Kowalik
    Klinika Wad Wrodzonych Serca, Instytut Kardiologii, ul Alpejska 42, 04 628 Warszawa
    Kardiol Pol 65:1442-6; discussion 1447-8. 2007
    ..At least 50% of cases of ARVC are inherited...
  69. ncbi Evaluation and course of an unusual case of arrhythmogenic right ventricular dysplasia
    Jan Fritz
    Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Int J Cardiovasc Imaging 22:269-73. 2006
    ..In this report, we describe accurate evaluation by MR imaging confirmed by histopathologic findings as well as imaging progression of this unusual case of arrhythmogenic right ventricular dysplasia.
  70. ncbi Structural and functional assessment of arrhythmogenic right ventricular dysplasia/cardiomyopathy by multi-slice computed tomography: comparison with cardiovascular magnetic resonance
    Yen-Wen Wu
    Int J Cardiol 115:e118-21. 2007
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an uncommon inheritable cardiomyopathy involving predominant right ventricle with progressive fibrofatty tissue replacement...
  71. ncbi Successful surgical treatment of heart failure and ventricular tachycardia in a patient with arrhythmogenic right ventricular dysplasia with cardiomyopathy
    Hidenori Sako
    Department of Cardiovascular Surgery, Oita University, School of Medicine, Oita, Japan
    Circ J 69:996-9. 2005
    A 70-year-old male patient with arrhythmogenic right ventricular dysplasia/cardiomyopathy demonstrating frequent attacks of ventricular tachycardia (VT) as well as heart failure underwent surgical treatment...
  72. ncbi Diagnostic value of plasma levels of brain natriuretic peptide in arrhythmogenic right ventricular dysplasia
    K Matsuo
    Divisions of Cardiology, Department of Internal Medicine, National Cardiovascular Center, Osaka, Japan
    Circulation 98:2433-40. 1998
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by local or diffuse wall motion abnormalities in the right ventricle (RV), associated with recurrent ventricular tachycardia (VT) of RV origin...
  73. ncbi Arrhythmogenic right ventricular dysplasia:clinical study
    Grazyna Markiewicz-Łoskot
    Department of Pediatric Cardiology, Medical University of Silesia, Katowice, Poland
    Ann Noninvasive Electrocardiol 12:181-4. 2007
    b>Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue...
  74. ncbi Arrhythmogenic right ventricular cardiomyopathy with evidence of biventricular involvement
    Robin Nijveldt
    Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands
    CMAJ 176:1819-21. 2007
  75. ncbi Arrhythmogenic right ventricular dysplasia presenting as right ventricular outflow tract tachycardia
    Christian Sticherling
    Division of Cardiology, University Hospital Basel, Petersgraben 4, 4031 Basel, Switzerland
    Europace 7:345-7. 2005
    ..An implantable cardioverter defibrillator (ICD) was implanted and an appropriate shock was later delivered...
  76. ncbi Contrast echocardiography for perfusion in right ventricular cardiomyopathy
    Attila Nemes
    2nd Department of Medicine and Cardiology Centre, University of Szeged, Szeged, Hungary
    Eur J Echocardiogr 6:470-2. 2005
    ..The present case suggests the clinical role of contrast echocardiography showing perfusion abnormalities in patients with RV cardiomyopathy in the region of the fat depositions...
  77. ncbi Arrhythmogenic right ventricular dysplasia
    Hugh Calkins
    Baltimore, Maryland, USA
    Trans Am Clin Climatol Assoc 119:273-86; discussion 287-8. 2008
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV)...
  78. ncbi Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia
    Domenico Corrado
    Department of Cardiac, Thoracic, and Vascular Sciences, University of Padua Medical School, Padua, Italy
    J Am Coll Cardiol 51:731-9. 2008
    ....
  79. ncbi Long-term follow-up of the signal-averaged ECG in arrhythmogenic right ventricular cardiomyopathy: correlation with arrhythmic events and echocardiographic findings
    Antonio Franco Folino
    Department of Cardiology, University of Padua, Padua, Italy
    Europace 8:423-9. 2006
    ....
  80. ncbi Use of a coronary sinus lead and biventricular ICD to correct a sensing abnormality in a patient with arrhythmogenic right ventricular dysplasia/cardiomyopathy
    Kenneth C Bilchick
    Division of Cardiology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
    J Cardiovasc Electrophysiol 17:317-20. 2006
    Implantable cardioverter defibrillators (ICDs) are frequently offered to patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)...
  81. ncbi Diagnosing subtle forms of potentially life-threatening diseases
    David J Callans
    J Am Coll Cardiol 51:740-1. 2008
  82. ncbi Differentiating arrhythmogenic right ventricular cardiomyopathy from right ventricular outflow tract ventricular tachycardia using multilead QRS duration and axis
    Craig D Ainsworth
    Division of Cardiology, University of Western Ontario, London, Canada
    Heart Rhythm 3:416-23. 2006
    ..CONCLUSION: QRS duration is longer in ARVC compared with RVOT VT. An algorithm combining lead I QRS duration for sensitivity and axis for specificity is useful for differentiating the two tachycardia substrates...
  83. ncbi Ventricular tachycardia in arrhythmogenic right ventricular dysplasia/cardiomyopathy: clinical presentation, risk stratification and results of long-term follow-up
    Thomas Pezawas
    Department of Cardiology, Medical University of Vienna, Waehringer Guertel 18 20, A 1090 Vienna, Austria
    Int J Cardiol 107:360-8. 2006
    Not all patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) are at risk for sudden cardiac death. The aim of the study was to evaluate the risk stratification in patients with ARVD/C.
  84. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy: clinical profile of four patients and review
    Lakshminarayan Yerra
    Cardiology Section, Department of Medicine, Louisiana State University Health Science Center, Shreveport, LA 71130, USA
    South Med J 101:309-16. 2008
    Four patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) are described. Two patients presented with sustained ventricular tachycardia and two with cardiac arrest from which they were successfully resuscitated...
  85. ncbi A pediatric case report on arrhythmogenic right ventricular dysplasia
    Ellen L P Chan
    UCLA School of Medicine, Mattel Children s Hospital Pediatric Cardiology, Los Angeles, CA 90015 9524, USA
    Congenit Heart Dis 3:132-7. 2008
    It is important for pediatric cardiologists to remember arrhythmogenic right ventricular dysplasia (ARVD) in the differential diagnosis of syncope in the pediatric population...
  86. ncbi [A heart insufficiency treated by glucocorticoids]
    L Froidevaux
    Service de Medecine Interne, Departement de Medecine Interne, Centre Hospitalier Universitaire Vaudois, Lausanne
    Praxis (Bern 1994) 96:1643-7. 2007
    b>Arrhythmogenic right ventricular dysplasia was diagnosed in 2000 in this 44-year-old male patient with a history of syncope. An internal defibrillator was implanted...
  87. ncbi Serial reevaluation for ARVD/C is indicated in patients presenting with left bundle branch block ventricular tachycardia and minor ECG abnormalities
    Philippine Kies
    Leiden University Medical Center, Leiden, The Netherlands
    J Cardiovasc Electrophysiol 17:586-93. 2006
    Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is based on a set of criteria proposed by the International Task Force (TF) for Cardiomyopathies in 1994...
  88. ncbi Ventricular arrhythmias in heart failure patients
    Ronald Lo
    Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford University Medical Center, Stanford, CA 94305 5233, USA
    Cardiol Clin 26:381-403, vi. 2008
    ....
  89. ncbi [Ventricular tachyarrhythmias in patients with cardiomyopathy]
    Kristoffer Henningsen
    Kardiologisk Klinik B, Hjertecentret, Rigshospitalet, DK 2100 København Ø
    Ugeskr Laeger 170:2238-42. 2008
    ..The purpose of this study was to determine the number and distribution of cardiomyopathies as the aetiology of ventricular tachyarrhythmias among patients discharged from the Department of Cardiology, Rigshospitalet...
  90. ncbi [MR imaging in cardiomyopathies]
    S Miller
    Radiologische Universitätsklinik Tübingen
    Rofo 177:1497-505. 2005
    ..This article describes characteristic aspects of MR imaging for the diagnosis of primary and secondary cardiomyopathies...
  91. ncbi Troponin-I elevation in a young man with arrhythmogenic right ventricular dysplasia/cardiomyopathy
    William J Kostis
    Department of Medicine, Johns Hopkins University School of Medicine, 600 North Wolfe Street, Baltimore, MD 21287, USA
    J Interv Card Electrophysiol 22:49-53. 2008
    Ventricular arrhythmias occur frequently in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) as well as those with ischemic heart disease...
  92. ncbi Three-dimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia
    Domenico Corrado
    Division of Cardiology, University of Padua Medical School, Padua, Italy
    Circulation 111:3042-50. 2005
    ....
  93. ncbi Pronounced epsilon waves in arrhythmogenic right ventricular dysplasia
    Peter Alter
    Department of Internal Medicine-Cardiology, Philipps University of Marburg/Lahn, Germany
    J Cardiovasc Electrophysiol 15:248. 2004
  94. ncbi [The arrhythmogenic right ventricular cardiomyopathy (ARVC)]
    K Wellemeyer
    , Ulmenallee 11, 49214 Bad Rothenfelde, Germany
    Z Kardiol 92:1033-8. 2003
    ..Furthermore, the present example offers the possibility of demonstrating that in the case of an ARVC in the regions of the right ventricle, arrhythmogenic areals can also be found which seem morphologically inconspicuous...
  95. ncbi T-wave alternans in patients with right ventricular tachycardia
    Osamu Kinoshita
    First Department of Internal Medicine, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    Cardiology 100:86-92. 2003
    ..Of the 7 patients with ARVC, 6 (86%) were positive for T-wave alternans. On the other hand, only 1 (8%) of 13 patients with idiopathic VT originating from the right-ventricular outflow tract was positive for T-wave alternans...
  96. ncbi Arrhythmogenic right ventricular dysplasia/cardiomyopathy in a Siberian husky
    M J Fernandez del Palacio
    Departamento de Patologia Animal, Facultad de Veterinaria, Universidad de Murcia, Espinardo, Spain
    J Small Anim Pract 42:137-42. 2001
    ..The right atrium and left ventricle were less severely affected by the same lesions. The clinical and pathological findings are similar to those reported in young people with arrhythmogenic right ventricular dysplasia/cardiomyopathy.
  97. ncbi [Arrhythmogenic right ventricular cardiomyopathy (dysplasia): etiology, clinical presentation, diagnosis and treatment]
    Elzbieta Czarnowska
    Instytut Pomnik - Centrum Zdrowia Dziecka, , Warszawa, Poland
    Kardiol Pol 58:58-63. 2003
    ..Treatment modalities including drug therapy, ablation, implantable cardioverter defibrillators, antiarrhythmic surgery or heart transplantation are also presented...
  98. ncbi [Right ventricular cardiomyopathy: a diagnosis to bear in mind]
    A Dominguez Rodriguez
    Servicio de Cardiologia, Hospital Universitario de Canarias, La Laguna, Santa Cruz de Tenerife
    An Med Interna 18:323-5. 2001
    ..We present the case of a 65-year-old male patient with the diagnosis of chronic liver disease, whose study lead a diagnosis of Arrhythmogenic right ventricular cardiomyopathy...
  99. ncbi [Arrhythmogenic right ventricular dysplasia/cardiomyopathy - diagnosis in childhood]
    T Kriebel
    , Germany
    Z Kardiol 92:418-24. 2003
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare, but important cause for sudden death in adolescents and young adults. Part of the patients affected show the pattern of autosomal-dominant inheritance...
  100. ncbi Arrhythmogenic right ventricular cardiomyopathy
    C Gemayel
    Hartford Hospital, Division of Cardiology, Hartford, Connecticut, USA
    J Am Coll Cardiol 38:1773-81. 2001
    ..This article will present in detail the etiology, clinical presentation, diagnosis and management of this condition...
  101. ncbi Cardiac sarcoidosis masquerading as right ventricular dysplasia
    Peter Ott
    University of Arizona Health Sciences Center, Tucson, Arizona 85724, USA
    Pacing Clin Electrophysiol 26:1498-503. 2003
    Patients with cardiac sarcoidosis may present with clinical and morphological features similar to arrhythmogenic right ventricular dysplasia (ARVD) or cardiomyopathy (ARVC)...

Research Grants78

  1. Multidisciplinary Study of Right Ventricular Dysplasia
    Wojciech Zareba; Fiscal Year: 2005
    ..multicenter, collaborative study to investigate the cardiac, clinical, and genetic aspects of arrhythmogenic right ventricular dysplasia (ARVD), a progressive disorder that predominantly affects the right side of the heart and causes ..
  2. Maturation of Normal and Disease-specific Human Stem Cell-derived Cardiomyocytes
    Huei Sheng Vincent Chen; Fiscal Year: 2010
    ..the first cardiac disease-specific iPSC line that produced CMs with pathological signatures of arrhythmogenic right ventricular dysplasia (ARVD)...
  3. A Clinical & Molecular Analysis of the Brugada Syndrome
    Barry London; Fiscal Year: 2003
    ..we identified two other large families and several small families with Brugada syndrome or arrhythmogenic right ventricular dysplasia (ARVD)...
  4. The molecular mechanisms underlying arrhythmogenic right ventricular dysplasia/ca
    Farah Sheikh; Fiscal Year: 2010
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic form of cardiomyopathy, which is typically characterized by right but also recently left ventricular dysfunction, fibrotic/fatty replacement of the ventricle ..
  5. The molecular mechanisms underlying arrhythmogenic right ventricular dysplasia/ca
    Farah Sheikh; Fiscal Year: 2010
    b>Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic form of cardiomyopathy, which is typically characterized by right but also recently left ventricular dysfunction, fibrotic/fatty replacement of the ..
  6. A Clinical & Molecular Analysis of the Brugada Syndrome
    Barry London; Fiscal Year: 2005
    ..we identified two other large families and several small families with Brugada syndrome or arrhythmogenic right ventricular dysplasia (ARVD)...
  7. Role of Desmosomal Adhesion in Carcinogenesis
    MY MAHONEY; Fiscal Year: 2009
    ..In human, genetic mutations in the DSG2 gene results in inherited arrhythmogenic right ventricular dysplasia/cardiomyopathy...
  8. Role of Desmosomal Adhesion in Carcinogenesis
    MY GEORGIA MAHONEY; Fiscal Year: 2011
    ..In human, genetic mutations in the DSG2 gene results in inherited arrhythmogenic right ventricular dysplasia/cardiomyopathy...
  9. Genetic Investigation of Arrhythmogenic Right Ventricular Dysplasia
    Daniel Judge; Fiscal Year: 2007
    ..of novel approaches to understand the molecular genetic basis for a rare cardiac disease, Arrhythmogenic Right Ventricular Dysplasia (ARVD)...
  10. CLONING OF A POTENTIAL REGULATOR OF THE DHAND FACTOR
    Mark Russell; Fiscal Year: 2002
    ..an inherited developmental abnormality of ventricular structure and electrophysiologic function, arrhythmogenic right ventricular dysplasia (ARVD)...
  11. Pediatric Cardiomyopathy Specimen Repository
    Jeffrey Towbin; Fiscal Year: 2007
    ....
  12. Multidisciplinary Study of Right Ventricular Dysplasia
    Jeffrey Towbin; Fiscal Year: 2005
    ..multicenter, collaborative study to investigate the cardiac, clinical, and genetic aspects of arrhythmogenic right ventricular dysplasia (ARVD), a progressive disorder that predominantly affects the right side of the heart and causes ..
  13. ELECTRICAL HETEROGENEITY AND CARDIAC ARRHYTHMIAS
    Charles Antzelevitch; Fiscal Year: 2007
    ..Our long-range goal is to generate information that will contribute meaningfully to our understanding of arrhythmic death in infants and young children. ..
  14. ELECTRICAL HETEROGENEITY AND CARDIAC ARRHYTHMIAS
    Charles Antzelevitch; Fiscal Year: 2000
    ..The long range goal is to generate information that will contribute to a more definitive and less empiric approach in the medical management of cardiac arrhythmias and sudden cardiac death. ..
  15. ELECTRICAL HETEROGENEITY AND CARDIAC ARRHYTHMIAS
    Charles Antzelevitch; Fiscal Year: 1993
    ..Our long-range goal is to narrow the gap that currently exists in this area and to generate information that will contribute to a more definitive and less empiric approach in the medical management of cardiac arrhythmias...
  16. Development of Novel Approaches for the Pharmacologic Treatment of Atrial Fibrill
    Charles Antzelevitch; Fiscal Year: 2011
    ..Successful completion of the studies proposed in this competing renewal will significantly advance this goal and lead to the development of innovative and effective pharmacologic treatments for AF. ..
  17. ELECTRICAL HETEROGENEITY AND CARDIAC ARRHYTHMIAS
    Charles Antzelevitch; Fiscal Year: 2004
    ..Our long-range goal is to generate information that will contribute to our understanding of the causes for arrhythmic death in infants and young children. ..
  18. Familial Dilated Cardiomyopathy: Detection/Gene Mapping
    Ray Hershberger; Fiscal Year: 2009
    ..We aim to identify more of the disease genes, which will lead to greater understanding of the causes of human heart failure. ..
  19. Familial Dilated Cardiomyopathy: Detection/Gene Mapping
    Ray E Hershberger; Fiscal Year: 2010
    ..We aim to identify more of the disease genes, which will lead to greater understanding of the causes of human heart failure. ..
  20. Update and modification of Task Force Criteria for ARVD/C
    Frank Marcus; Fiscal Year: 2007
    b>Arrhythmogenic Right Ventricular Dysplasia /Cardiomyopathy (ARVD/C) is a disease characterized by a presence of ventricular arrhythmias originating from the right ventricle including ventricular premature beats, ventricular tachycardia or ..
  21. FAMILIAL DILATED CARDIOMYOPATHY--DETECTION/GENE MAPPING
    Ray Hershberger; Fiscal Year: 2000
    ..Preliminary data suggest that OHSU FDC-1 links to neither of the recently reported cardiomyopathy chromosomal locations, suggesting that an additional locus for FDC is present. ..
  22. Multidisciplinary Study of Right Ventricular Dysplasia
    Frank Marcus; Fiscal Year: 2005
    ..multicenter, collaborative study to investigate the cardiac, clinical, and genetic aspects of arrhythmogenic right ventricular dysplasia (ARVD), a progressive disorder that predominantly affects the right side of the heart and causes ..
  23. Detection of Lipid Infiltration in the Heart with MRI
    MARIA ALTBACH; Fiscal Year: 2009
    ..The main purpose of the project is to improve the diagnosis of a condition known as Arrhythmogenic Right Ventricular Dysplasia (ARVD) a disease characterized by fibrofatty infiltration in the right ventricle that leads to ..
  24. Genetic Predictors of QT Response During Exercise
    Prince Kannankeril; Fiscal Year: 2007
    ..This research has the potential to permit identification of individuals at risk for sudden cardiac death and therefore has significant relevance to public health. (End of Abstract) ..
  25. Detection of Lipid Infiltration in the Heart with MRI
    MARIA ALTBACH; Fiscal Year: 2007
    ..The main purpose of the project is to improve the diagnosis of a condition known as Arrhythmogenic Right Ventricular Dysplasia (ARVD) a disease characterized by fibrofatty infiltration in the right ventricle that leads to ..
  26. Novel radial MRI methods for abdominal imaging
    MARIA ALTBACH; Fiscal Year: 2005
    ..0 T. The overall goal is to develop the acquisition and post-processing methods and then to show that these lead to improve detection and characterization of neoplasms. ..
  27. Free-Breathing 3D Cardiac MR Imaging
    Bruno Madore; Fiscal Year: 2005
    ..This approach has the potential of being especially useful in patients for whom breath-holding is not an option, e.g. when imaging very sick, mentally impaired or infant patients. ..
  28. Coronary Atherosclerosis Evaluation by Arterial Wall MRI
    David Bluemke; Fiscal Year: 2007
    ..The ability to directly define the extent of coronary atherosclerosis at an early stage should lead to an improved understanding of coronary risk factors and their impact on atherosclerosis development. ..
  29. Familial Dilated Cardiomyopathy: Detection/Gene Mapping
    Ray Hershberger; Fiscal Year: 2006
    ..We further propose to (2) map the genes responsible for FDC in several FDC pedigrees, of which linkage and additional gene mapping studies are in progress. ..
  30. CAUSE OF CARDIOVASCULAR COMPLICATIONS IN HIV INFECTION
    Jeffrey Towbin; Fiscal Year: 2002
    ....
  31. IDENTIFICATION OF THE GENES CAUSING BRUGADA SYNDROME
    Jeffrey Towbin; Fiscal Year: 2002
    ..If pathogenic mutations are found, he proposes to collaborate in expressing them in frog oocytes to study the effect of the mutations on ion fluxes and action potentials. ..