Arrhythmogenic phenotype in dilated cardiomyopathy: Natural history and predictors of life-threatening arrhythmias

Anita Spezzacatene, Gianfranco Sinagra, Marco Merlo, Giulia Barbati, Sharon L. Graw, Francesca Brun, Dobromir Slavov, Andrea Di Lenarda, Ernesto E. Salcedo, Jeffrey Towbin, Jeffrey E. Saffitz, Frank I. Marcus, Wojciech Zareba, Matthew R.G. Taylor, Luisa Mestroni

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Abstract

Background-Patients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (ARDCM). We investigated the phenotype and natural history of patients with AR-DCM. Methods and Results-Two hundred eighty-five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR-DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2%) met criteria for AR-DCM phenotype. AR-DCM subjects had a higher incidence of SCD/SVT/VF compared with non-AR-DCM patients (30.3% vs 17.6%, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR-DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF. Conclusions-One-third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow-up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.

Original languageEnglish (US)
Article numbere002149
JournalJournal of the American Heart Association
Volume4
Issue number10
DOIs
StatePublished - Jan 1 2015

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Dilated Cardiomyopathy
Natural History
Cardiac Arrhythmias
Phenotype
Sudden Cardiac Death
Ventricular Tachycardia
Ventricular Fibrillation
Heart Failure
Ambulatory Electrocardiography
Ventricular Premature Complexes
Syncope
Left Ventricular Dysfunction
Heart Transplantation

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

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Arrhythmogenic phenotype in dilated cardiomyopathy : Natural history and predictors of life-threatening arrhythmias. / Spezzacatene, Anita; Sinagra, Gianfranco; Merlo, Marco; Barbati, Giulia; Graw, Sharon L.; Brun, Francesca; Slavov, Dobromir; Di Lenarda, Andrea; Salcedo, Ernesto E.; Towbin, Jeffrey; Saffitz, Jeffrey E.; Marcus, Frank I.; Zareba, Wojciech; Taylor, Matthew R.G.; Mestroni, Luisa.

In: Journal of the American Heart Association, Vol. 4, No. 10, e002149, 01.01.2015.

Research output: Contribution to journalArticle

Spezzacatene, A, Sinagra, G, Merlo, M, Barbati, G, Graw, SL, Brun, F, Slavov, D, Di Lenarda, A, Salcedo, EE, Towbin, J, Saffitz, JE, Marcus, FI, Zareba, W, Taylor, MRG & Mestroni, L 2015, 'Arrhythmogenic phenotype in dilated cardiomyopathy: Natural history and predictors of life-threatening arrhythmias', Journal of the American Heart Association, vol. 4, no. 10, e002149. https://doi.org/10.1161/JAHA.115.002149
Spezzacatene, Anita ; Sinagra, Gianfranco ; Merlo, Marco ; Barbati, Giulia ; Graw, Sharon L. ; Brun, Francesca ; Slavov, Dobromir ; Di Lenarda, Andrea ; Salcedo, Ernesto E. ; Towbin, Jeffrey ; Saffitz, Jeffrey E. ; Marcus, Frank I. ; Zareba, Wojciech ; Taylor, Matthew R.G. ; Mestroni, Luisa. / Arrhythmogenic phenotype in dilated cardiomyopathy : Natural history and predictors of life-threatening arrhythmias. In: Journal of the American Heart Association. 2015 ; Vol. 4, No. 10.
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title = "Arrhythmogenic phenotype in dilated cardiomyopathy: Natural history and predictors of life-threatening arrhythmias",
abstract = "Background-Patients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (ARDCM). We investigated the phenotype and natural history of patients with AR-DCM. Methods and Results-Two hundred eighty-five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR-DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2{\%}) met criteria for AR-DCM phenotype. AR-DCM subjects had a higher incidence of SCD/SVT/VF compared with non-AR-DCM patients (30.3{\%} vs 17.6{\%}, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR-DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF. Conclusions-One-third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow-up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.",
author = "Anita Spezzacatene and Gianfranco Sinagra and Marco Merlo and Giulia Barbati and Graw, {Sharon L.} and Francesca Brun and Dobromir Slavov and {Di Lenarda}, Andrea and Salcedo, {Ernesto E.} and Jeffrey Towbin and Saffitz, {Jeffrey E.} and Marcus, {Frank I.} and Wojciech Zareba and Taylor, {Matthew R.G.} and Luisa Mestroni",
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T1 - Arrhythmogenic phenotype in dilated cardiomyopathy

T2 - Natural history and predictors of life-threatening arrhythmias

AU - Spezzacatene, Anita

AU - Sinagra, Gianfranco

AU - Merlo, Marco

AU - Barbati, Giulia

AU - Graw, Sharon L.

AU - Brun, Francesca

AU - Slavov, Dobromir

AU - Di Lenarda, Andrea

AU - Salcedo, Ernesto E.

AU - Towbin, Jeffrey

AU - Saffitz, Jeffrey E.

AU - Marcus, Frank I.

AU - Zareba, Wojciech

AU - Taylor, Matthew R.G.

AU - Mestroni, Luisa

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Background-Patients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (ARDCM). We investigated the phenotype and natural history of patients with AR-DCM. Methods and Results-Two hundred eighty-five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR-DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2%) met criteria for AR-DCM phenotype. AR-DCM subjects had a higher incidence of SCD/SVT/VF compared with non-AR-DCM patients (30.3% vs 17.6%, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR-DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF. Conclusions-One-third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow-up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.

AB - Background-Patients with dilated cardiomyopathy (DCM) may present with ventricular arrhythmias early in the disease course, unrelated to the severity of left ventricular dysfunction. These patients may be classified as having an arrhythmogenic DCM (ARDCM). We investigated the phenotype and natural history of patients with AR-DCM. Methods and Results-Two hundred eighty-five patients with a recent diagnosis of DCM (median duration of the disease 1 month, range 0 to 7 months) and who had Holter monitoring at baseline were comprehensively evaluated and followed for 107 months (range 29 to 170 months). AR-DCM was defined by the presence of ≥1 of the following: unexplained syncope, rapid nonsustained ventricular tachycardia (≥5 beats, ≥150 bpm), ≥1000 premature ventricular contractions/24 hours, and ≥50 ventricular couplets/24, in the absence of overt heart failure. The primary end points were sudden cardiac death (SCD), sustained ventricular tachycardia (SVT), or ventricular fibrillation (VF). The secondary end points were death from congestive heart failure or heart transplantation. Of the 285 patients, 109 (38.2%) met criteria for AR-DCM phenotype. AR-DCM subjects had a higher incidence of SCD/SVT/VF compared with non-AR-DCM patients (30.3% vs 17.6%, P=0.022), with no difference in the secondary end points. A family history of SCD/SVT/VF and the AR-DCM phenotype were statistically significant and cumulative predictors of SCD/SVT/VF. Conclusions-One-third of DCM patients may have an arrhythmogenic phenotype associated with increased risk of arrhythmias during follow-up. A family history of ventricular arrhythmias in DCM predicts a poor prognosis and increased risk of SCD.

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