Current concepts on diagnosis and prognosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia

Sandra L.Castaños Gutiérrez, Ihab R. Kamel, Stefan L. Zimmerman

Research output: Contribution to journalArticle

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an uncommon cardiac disease characterized by progressive right ventricular dysfunction due to fibrofatty replacement of myocytes and risk of sudden cardiac death from malignant arrhythmias. ARVC/D is a disease of the cardiac desmosome, with genetic mutations in genes encoding proteins critical to this structure found in the majority of patients. The diagnosis of ARVC/D is based on fulfilling a combination of clinical, imaging, pathologic, and/or genetic criteria set forth by the 2010 modified Task Force Criteria. Cardiac magnetic resonance (CMR) is included in these criteria and plays an important role in the management of ARVC/D, demonstrating pathologic structural changes in the right and left ventricles that provide both diagnostic and prognostic information. The purpose of this article is to provide a background on the pathophysiology and genetics of ARVC/D and focus on the role of CMR in management of ARVC/D including diagnosis, prognosis, and treatment decisions. Common CMR pitfalls that can lead to misdiagnosis will also be reviewed.

Original languageEnglish (US)
Pages (from-to)324-335
Number of pages12
JournalJournal of Thoracic Imaging
Volume31
Issue number6
DOIs
StatePublished - Nov 1 2016

Keywords

  • Task Force Criteria
  • arrhythmogenic right ventricular cardiomyopathy
  • cardiac magnetic resonance
  • diagnosis
  • pitfalls
  • prognosis

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Pulmonary and Respiratory Medicine

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