Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy- associated desmosomal mutation carriers

Anneline S J M Te Riele, Aditya Bhonsale, Cynthia Anne James, Neda Rastegar, Brittney Murray, Jeremy R. Burt, Crystal Tichnell, Srinivasa Madhavan, Daniel P. Judge, David A. Bluemke, Stefan Zimmerman, Ihab R Kamel, Hugh Calkins, Harikrishna Tandri

Research output: Contribution to journalArticle

Abstract

Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 ± 15.3 years, 42% men) harboring ARVD/C-associated pathogenic mutations (83% plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61%) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48%) had abnormal results on CMR. Only 1 of 27 patients (4%) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 ± 4.4 years, 11 patients (16%) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement.

Original languageEnglish (US)
Pages (from-to)1761-1769
Number of pages9
JournalJournal of the American College of Cardiology
Volume62
Issue number19
DOIs
StatePublished - Nov 5 2013

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Arrhythmogenic Right Ventricular Dysplasia
Magnetic Resonance Imaging
Ambulatory Electrocardiography
Magnetic Resonance Spectroscopy
Mutation
Cardiac Arrhythmias
Electrocardiography
Advisory Committees
Plakophilins
Implantable Defibrillators

Keywords

  • cardiomyopathy
  • electrocardiography
  • magnetic resonance imaging
  • risk stratification
  • tachyarrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy- associated desmosomal mutation carriers. / Te Riele, Anneline S J M; Bhonsale, Aditya; James, Cynthia Anne; Rastegar, Neda; Murray, Brittney; Burt, Jeremy R.; Tichnell, Crystal; Madhavan, Srinivasa; Judge, Daniel P.; Bluemke, David A.; Zimmerman, Stefan; Kamel, Ihab R; Calkins, Hugh; Tandri, Harikrishna.

In: Journal of the American College of Cardiology, Vol. 62, No. 19, 05.11.2013, p. 1761-1769.

Research output: Contribution to journalArticle

Te Riele, Anneline S J M ; Bhonsale, Aditya ; James, Cynthia Anne ; Rastegar, Neda ; Murray, Brittney ; Burt, Jeremy R. ; Tichnell, Crystal ; Madhavan, Srinivasa ; Judge, Daniel P. ; Bluemke, David A. ; Zimmerman, Stefan ; Kamel, Ihab R ; Calkins, Hugh ; Tandri, Harikrishna. / Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy- associated desmosomal mutation carriers. In: Journal of the American College of Cardiology. 2013 ; Vol. 62, No. 19. pp. 1761-1769.
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abstract = "Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 ± 15.3 years, 42{\%} men) harboring ARVD/C-associated pathogenic mutations (83{\%} plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61{\%}) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48{\%}) had abnormal results on CMR. Only 1 of 27 patients (4{\%}) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 ± 4.4 years, 11 patients (16{\%}) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement.",
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T1 - Incremental value of cardiac magnetic resonance imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy- associated desmosomal mutation carriers

AU - Te Riele, Anneline S J M

AU - Bhonsale, Aditya

AU - James, Cynthia Anne

AU - Rastegar, Neda

AU - Murray, Brittney

AU - Burt, Jeremy R.

AU - Tichnell, Crystal

AU - Madhavan, Srinivasa

AU - Judge, Daniel P.

AU - Bluemke, David A.

AU - Zimmerman, Stefan

AU - Kamel, Ihab R

AU - Calkins, Hugh

AU - Tandri, Harikrishna

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N2 - Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 ± 15.3 years, 42% men) harboring ARVD/C-associated pathogenic mutations (83% plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61%) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48%) had abnormal results on CMR. Only 1 of 27 patients (4%) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 ± 4.4 years, 11 patients (16%) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement.

AB - Objectives The aim of this study was to identify the incremental value and optimal role of cardiac magnetic resonance (CMR) imaging in arrhythmic risk stratification of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C)-associated desmosomal mutation carriers without histories of sustained ventricular arrhythmia. Background Risk stratification of ARVD/C mutation carriers is challenging. Methods Sixty-nine patients (mean age 27.0 ± 15.3 years, 42% men) harboring ARVD/C-associated pathogenic mutations (83% plakophilin 2) without prior sustained ventricular arrhythmias were included. Electrocardiographic and 24-h Holter monitoring findings closest to presentation were analyzed for electrical abnormalities per revised task force criteria. CMR studies were done to identify abnormal cardiac structure and function according to the revised task force criteria. Results Overall, 42 patients (61%) presented with electrical abnormalities on the basis of electrocardiography and Holter monitoring, of whom 20 (48%) had abnormal results on CMR. Only 1 of 27 patients (4%) without electrical abnormalities at initial evaluation had abnormal CMR results. Over a mean follow-up period of 5.8 ± 4.4 years, 11 patients (16%) experienced sustained ventricular arrhythmias, exclusively in patients with both electrical abnormalities (electrocardiography and/or Holter monitoring) and abnormal CMR results. Conclusions These results suggest that electrical abnormalities on electrocardiography and Holter monitoring precede detectable structural abnormalities in ARVD/C mutation carriers. Therefore, evaluation of cardiac structure and function using CMR is probably not necessary in the absence of baseline electrical abnormalities. Among ARVD/C mutation carriers, the presence of both electrical and CMR abnormalities identifies patients at high risk for events and thus patients who might benefit from prophylactic implantable cardioverter-defibrillator placement.

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KW - tachyarrhythmias

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