Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: results

Frank I Marcus1, Wojciech Zareba, Hugh Calkins

  • 1Section of Cardiology, University of Arizona, 1501 N. Campbell Avenue, Tucson, Arizona 85724-0001, USA. fmarcus@email.arizona.edu

Heart Rhythm
|June 30, 2009
PubMed

Insights

Diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) in newly identified patients requires multiple tests. Combining echocardiography, angiography, ECG, and Holter monitoring aids accurate ARVC/D evaluation.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Limited data exists on diagnostic performance for early arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) cases.
  • Previous ARVC/D research focused on advanced disease stages.

Purpose of the Study:

  • To characterize newly diagnosed ARVC/D patients.
  • To evaluate diagnostic modalities in a large ARVC/D cohort.

Main Methods:

  • 108 newly diagnosed ARVC/D patients prospectively enrolled.
  • Standardized noninvasive and invasive testing with core laboratory adjudication.
  • Mean follow-up of 27 months.

Main Results:

  • Newly diagnosed ARVC/D patients present differently than those with advanced disease.
  • Significant discrepancies noted between initial and final ARVC/D classifications.
  • Individual diagnostic tests showed 50-70% agreement with final diagnosis in affected patients.

Conclusions:

  • A combination of diagnostic tests is essential for evaluating ARVC/D.
  • Echocardiography, right ventricular angiography, signal-averaged ECG, and Holter monitoring are optimal for ARVC/D assessment.
Abstract

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