Malignant Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy with a normal 12-lead electrocardiogram: A rare but underrecognized clinical entity

A.S.J.M. te Riele, C.A. James, A. Bhonsale, J.A. Groeneweg, C.F. Camm, B. Murray, C. Tichnell, J.F. van der Heijden, D. Dooijes, D.P. Judge, R.N.W. Hauer, H. Tandri, H. Calkins

Research output: Contribution to journalArticleAcademicpeer-review

9 Citations (Scopus)

Abstract

BACKGROUND In Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), a normal electrocardiogram (ECG) is considered reassuring. However, some patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG.

OBJECTIVES To estimate how often patients with ARVD/C experiencing ventricular arrhythmias have a normal ECG during sinus rhythm, and to provide a clinical profile of these patients.

METHODS We included 145 patients with ARVD/C experiencing a documented sustained ventricular arrhythmia. Conventional 12-lead sinus rhythm ECGs within 6 months of the event were reviewed for diagnostic Task Force Criteria (TFC). ECGs were classified as abnormal (>= 1 TFC), nonspecific (abnormal, no TFC), or normal. Cardiologic investigations within 6 months of the event were evaluated as per TFC in those with a nonspecific or normal ECG.

RESULTS The ECG was nonspecific or normal in 17 of 145 (12%) subjects. Mean age of these patients was 41.3 12.4 years and 14 (82%) were men, comparable to those with an abnormal ECG. Most patients with a nonspecific or normal ECG showed >= 1 TFC on Hotter monitoring (n = 9 of 10) and signal-averaged ECG (n = 4 of 5), and all had nonsustained ventricular tachycardia recorded. Among 15 patients who underwent structural evaluation, 11 (73%) showed structural TFC (9 major and 2 minor).

CONCLUSIONS Although most patients with ARVD/C experiencing arrhythmias have an abnormal ECG, a nonspecific or normal ECG does not preclude ARVD/C diagnosis. All patients with a nonspecific or normal ECG had alternative evidence of disease expression. These results alert the physician not to rely exclusively on ECG in ARVD/C, but to assess arrhythmic risk by comprehensive clinical evaluation.

Original languageEnglish
Pages (from-to)1484-1491
Number of pages8
JournalHeart Rhythm
Volume10
Issue number10
DOIs
Publication statusPublished - Oct 2013

Keywords

  • Cardiomyopathy
  • Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
  • Electrocardiogram
  • Risk stratification
  • Diagnosis
  • Concealed
  • Ventricular arrhythmias
  • ST-SEGMENT ELEVATION
  • TASK-FORCE CRITERIA
  • BUNDLE-BRANCH BLOCK
  • BRUGADA-SYNDROME
  • DYSPLASIA
  • CARDIOMYOPATHY/DYSPLASIA
  • MULTICENTER
  • DIAGNOSIS
  • DEATH

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