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

Anneline S J M Te Riele, Cynthia Anne James, Aditya Bhonsale, Judith A. Groeneweg, Christian F. Camm, Brittney Murray, Crystal Tichnell, Jeroen F. Van Der Heijden, Dennis Dooijes, Daniel P. Judge, Richard N W Hauer, Harikrishna Tandri, Hugh Calkins

Research output: Contribution to journalArticle

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 Holter 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 (US)
Pages (from-to)1484-1491
Number of pages8
JournalHeart Rhythm
Volume10
Issue number10
DOIs
StatePublished - Oct 2013

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Arrhythmogenic Right Ventricular Dysplasia
Electrocardiography
Advisory Committees
Cardiac Arrhythmias
Lead
Ambulatory Electrocardiography
Ventricular Tachycardia

Keywords

  • Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
  • Cardiomyopathy
  • Concealed
  • Diagnosis
  • Electrocardiogram
  • Riskstratification
  • Ventriculararrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Malignant Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy with a normal 12-lead electrocardiogram : A rare but underrecognized clinical entity. / Te Riele, Anneline S J M; James, Cynthia Anne; Bhonsale, Aditya; Groeneweg, Judith A.; Camm, Christian F.; Murray, Brittney; Tichnell, Crystal; Van Der Heijden, Jeroen F.; Dooijes, Dennis; Judge, Daniel P.; Hauer, Richard N W; Tandri, Harikrishna; Calkins, Hugh.

In: Heart Rhythm, Vol. 10, No. 10, 10.2013, p. 1484-1491.

Research output: Contribution to journalArticle

Te Riele, ASJM, James, CA, Bhonsale, A, Groeneweg, JA, Camm, CF, Murray, B, Tichnell, C, Van Der Heijden, JF, Dooijes, D, Judge, DP, Hauer, RNW, Tandri, H & Calkins, H 2013, 'Malignant Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy with a normal 12-lead electrocardiogram: A rare but underrecognized clinical entity', Heart Rhythm, vol. 10, no. 10, pp. 1484-1491. https://doi.org/10.1016/j.hrthm.2013.06.022
Te Riele, Anneline S J M ; James, Cynthia Anne ; Bhonsale, Aditya ; Groeneweg, Judith A. ; Camm, Christian F. ; Murray, Brittney ; Tichnell, Crystal ; Van Der Heijden, Jeroen F. ; Dooijes, Dennis ; Judge, Daniel P. ; Hauer, Richard N W ; Tandri, Harikrishna ; Calkins, Hugh. / Malignant Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy with a normal 12-lead electrocardiogram : A rare but underrecognized clinical entity. In: Heart Rhythm. 2013 ; Vol. 10, No. 10. pp. 1484-1491.
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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 Holter 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.",
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AU - James, Cynthia Anne

AU - Bhonsale, Aditya

AU - Groeneweg, Judith A.

AU - Camm, Christian F.

AU - Murray, Brittney

AU - Tichnell, Crystal

AU - Van Der Heijden, Jeroen F.

AU - Dooijes, Dennis

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N2 - 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 Holter 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.

AB - 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 Holter 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.

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KW - Electrocardiogram

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