Diagnosing arrhythmogenic right ventricular cardiomyopathy by 2010 task force criteria: Clinical performance and simplified practical implementation

Laurens P. Bosman, Julia Cadrin-Tourigny, Mimount Bourfiss, Mounes Aliyari Ghasabeh, Apurva Sharma, Crystal Tichnell, Rob W. Roudijk, Brittney Murray, Harikrishna Tandri, Paul Khairy, Ihab R. Kamel, Stefan L. Zimmerman, Johannes B. Reitsma, Folkert W. Asselbergs, J. Peter van Tintelen, Jeroen F. van der Heijden, Richard N.W. Hauer, Hugh Calkins, Cynthia A. James, Anneline S.J.M. te Riele

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is diagnosed by a complex set of clinical tests as per 2010 Task Force Criteria (TFC). Avoiding misdiagnosis is crucial to prevent sudden cardiac death as well as unnecessary implantable cardioverter-defibrillator implantations. This study aims to validate the overall performance of the TFC in a real-world cohort of patients referred for ARVC evaluation. Methods We included patients consecutively referred to our centres for ARVC evaluation. Patients were diagnosed by con- and results sensus of three independent clinical experts. Using this as a reference standard, diagnostic performance was measured for each individual criterion as well as the overall TFC classification. Of 407 evaluated patients (age 38 ± 17 years, 51% male), the expert panel diagnosed 66 (16%) with ARVC. The clinically observed TFC was false negative in 7/66 (11%) patients and false positive in 10/69 (14%) patients. Idiopathic outflow tract ventricular tachycardia was the most common alternative diagnosis. While the TFC performed well overall (sensitivity and specificity 92%), signal-averaged electrocardiogram (SAECG, P = 0.43), and several family history criteria (P >_ 0.17) failed to discriminate. Eliminating these criteria reduced false positives without increasing false negatives (net reclassification improvement 4.3%, P = 0.019). Furthermore, all ARVC patients met at least one electrocardiogram (ECG) or arrhythmia criterion (sensitivity 100%). Conclusion The TFC perform well but are complex and can lead to misdiagnosis. Simplification by eliminating SAECG and several family history criteria improves diagnostic accuracy. Arrhythmogenic right ventricular cardiomyopathy can be ruled out using ECG and arrhythmia criteria alone, hence these tests may serve as a first-line screening strategy among at-risk individuals.

Original languageEnglish (US)
Pages (from-to)787-796
Number of pages10
Issue number5
StatePublished - May 1 2020


  • Arrhythmogenic right ventricular cardiomyopathy
  • Cardiomyopathy
  • Diagnosis
  • Ventricular arrhythmia

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


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