Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Hicham Z. El Masry, Anil V. Yadav

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiac disease characterized by fibrofatty replacement of the cardiac myocytes. Patients with ARVD/C frequently present with ventricular tachycardia and many are thought to have sudden cardiac death as the initial manifestation of the disease. Over the past decade, our understanding of the disease has increased dramatically along with delineation of the genetic basis of ARVD/C and characteristic features on diagnostic imaging. The management of patients with ARVD/C remains a challenge, especially in the light of incomplete genotype-phenotype characterization, and poor predictors of sudden cardiac death. In this article, we review the pathologic and genetic basis of ARVD/C, focusing on the diagnostic features and therapeutic challenges emerging with our enhanced knowledge of this rare disease.

Original languageEnglish
Pages (from-to)249-260
Number of pages12
JournalExpert Review of Cardiovascular Therapy
Volume6
Issue number2
DOIs
StatePublished - Feb 2008

Fingerprint

Arrhythmogenic Right Ventricular Dysplasia
Sudden Cardiac Death
Diagnostic Imaging
Ventricular Tachycardia
Rare Diseases
Cardiac Myocytes
Heart Diseases
Genotype
Phenotype

Keywords

  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Arrhythmogenic right ventricular dysplasia/cardiomyopathy. / El Masry, Hicham Z.; Yadav, Anil V.

In: Expert Review of Cardiovascular Therapy, Vol. 6, No. 2, 02.2008, p. 249-260.

Research output: Contribution to journalArticle

El Masry, Hicham Z. ; Yadav, Anil V. / Arrhythmogenic right ventricular dysplasia/cardiomyopathy. In: Expert Review of Cardiovascular Therapy. 2008 ; Vol. 6, No. 2. pp. 249-260.
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