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Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Kalpana R Prakasa1, Hugh Calkins

  • 1Carnegie 592, Johns Hopkins Hospital, 600, N. Wolfe Street, Baltimore, MD 21287, USA.

Current Treatment Options in Cardiovascular Medicine
|November 15, 2005
PubMed

Insights

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) diagnosis relies on established criteria, not solely MRI. Early identification and ICD implantation are crucial for managing ventricular arrhythmias and preventing sudden death in ARVD/C patients.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic heart condition causing ventricular arrhythmias and right ventricular dysfunction.
  • Accurate diagnosis is paramount for effective ARVD/C management.
  • Current diagnostic practices may overemphasize MRI, neglecting established International Task Force criteria.

Purpose of the Study:

  • To highlight the importance of International Task Force criteria for diagnosing ARVD/C.
  • To emphasize the typical noninvasive and invasive diagnostic findings in ARVD/C.
  • To guide treatment decisions, including ICD implantation and genetic screening.

Main Methods:

  • Review of diagnostic criteria for ARVD/C.
  • Discussion of noninvasive tests (ECG, echocardiogram) and invasive tests (RV angiogram, biopsy, EP study).
  • Consideration of implantable cardioverter-defibrillator (ICD) indications and pharmacologic treatments (beta-blockers, ACE inhibitors).
  • Emphasis on genetic screening for plakophilin-2 mutations.

Main Results:

  • ARVD/C diagnosis requires adherence to International Task Force criteria.
  • Patients typically present with abnormal ECG, echocardiogram, and ventricular arrhythmias.
  • Invasive testing is recommended when noninvasive findings are suggestive.
  • ICDs are indicated for syncope, sudden death, sustained ventricular arrhythmias, or specific ARVD/C evidence.
  • Beta blockers and ACE inhibitors are recommended treatments.
  • Plakophilin-2 mutation screening is beneficial for diagnosis and family management.

Conclusions:

  • Correct diagnosis of ARVD/C hinges on International Task Force criteria.
  • A comprehensive approach combining noninvasive, invasive, and genetic testing is essential.
  • Timely ICD implantation and appropriate medical therapy improve outcomes for ARVD/C patients.

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