Arrhythmogenic Phenotype in Dilated Cardiomyopathy: Natural History and Predictors of Life-Threatening Arrhythmias
- PMID: 26475296
- PMCID: PMC4845125
- DOI: 10.1161/JAHA.115.002149
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 (AR-DCM). 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 hours, 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.
Keywords: arrhythmia; cardiomyopathy; prognosis; sudden death.
© 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.
Figures
![Figure 1](https://cdn.statically.io/img/www.ncbi.nlm.nih.gov/pmc/articles/instance/4845125/bin/JAH3-4-e002149-g001.gif)
![Figure 2](https://cdn.statically.io/img/www.ncbi.nlm.nih.gov/pmc/articles/instance/4845125/bin/JAH3-4-e002149-g002.gif)
![Figure 3](https://cdn.statically.io/img/www.ncbi.nlm.nih.gov/pmc/articles/instance/4845125/bin/JAH3-4-e002149-g003.gif)
![Figure 4](https://cdn.statically.io/img/www.ncbi.nlm.nih.gov/pmc/articles/instance/4845125/bin/JAH3-4-e002149-g004.gif)
Similar articles
-
Role of arrhythmic phenotype in prognostic stratification and management of dilated cardiomyopathy.Eur J Heart Fail. 2024 Mar;26(3):581-589. doi: 10.1002/ejhf.3168. Epub 2024 Feb 26. Eur J Heart Fail. 2024. PMID: 38404225
-
Arrhythmic risk stratification in patients with dilated cardiomyopathy and intermediate left ventricular dysfunction.J Cardiovasc Med (Hagerstown). 2019 May;20(5):343-350. doi: 10.2459/JCM.0000000000000792. J Cardiovasc Med (Hagerstown). 2019. PMID: 30921270
-
A novel LMNA nonsense mutation causes two distinct phenotypes of cardiomyopathy with high risk of sudden cardiac death in a large five-generation family.Europace. 2018 Dec 1;20(12):2003-2013. doi: 10.1093/europace/euy127. Europace. 2018. PMID: 29947763
-
Genotype-phenotype associations in dilated cardiomyopathy: meta-analysis on more than 8000 individuals.Clin Res Cardiol. 2017 Feb;106(2):127-139. doi: 10.1007/s00392-016-1033-6. Epub 2016 Aug 30. Clin Res Cardiol. 2017. PMID: 27576561 Review.
-
Ventricular arrhythmias in dilated cardiomyopathy.Pacing Clin Electrophysiol. 1997 Oct;20(10 Pt 2):2714-8. doi: 10.1111/j.1540-8159.1997.tb06121.x. Pacing Clin Electrophysiol. 1997. PMID: 9358519 Review.
Cited by
-
Is Cardiac Transplantation Still a Contraindication in Patients with Muscular Dystrophy-Related End-Stage Dilated Cardiomyopathy? A Systematic Review.Int J Mol Sci. 2024 May 13;25(10):5289. doi: 10.3390/ijms25105289. Int J Mol Sci. 2024. PMID: 38791328 Free PMC article. Review.
-
Inherited Arrhythmias in the Pediatric Population: An Updated Overview.Medicina (Kaunas). 2024 Jan 3;60(1):94. doi: 10.3390/medicina60010094. Medicina (Kaunas). 2024. PMID: 38256355 Free PMC article. Review.
-
New perspectives in diagnosis and risk stratification of non-ischaemic dilated cardiomyopathy.Eur Heart J Suppl. 2023 Apr 26;25(Suppl C):C137-C143. doi: 10.1093/eurheartjsupp/suad016. eCollection 2023 May. Eur Heart J Suppl. 2023. PMID: 37125318 Free PMC article.
-
The electrocardiogram in non-ischaemic-dilated cardiomyopathy.Eur Heart J Suppl. 2023 Apr 26;25(Suppl C):C179-C184. doi: 10.1093/eurheartjsupp/suad043. eCollection 2023 May. Eur Heart J Suppl. 2023. PMID: 37125290 Free PMC article.
-
Dilated Cardiomyopathy: A Comprehensive Approach to Diagnosis and Risk Stratification.Biomedicines. 2023 Mar 9;11(3):834. doi: 10.3390/biomedicines11030834. Biomedicines. 2023. PMID: 36979813 Free PMC article. Review.
References
-
- Sen‐Chowdhry S, Syrris P, Prasad SK, Hughes SE, Merrifield R, Ward D, Pennell DJ, McKenna WJ. Left‐dominant arrhythmogenic cardiomyopathy: an under‐recognized clinical entity. J Am Coll Cardiol. 2008;52:2175–2187. - PubMed
-
- Sen‐Chowdhry S, Syrris P, Pantazis A, Quarta G, McKenna WJ, Chambers JC. Mutational heterogeneity, modifier genes, and environmental influences contribute to phenotypic diversity of arrhythmogenic cardiomyopathy. Circ Cardiovasc Genet. 2010;3:323–330. - PubMed
-
- Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A, Mahon NG, McKenna WJ. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol. 2000;36:2212–2218. - PubMed
-
- Ritter M, Oechslin E, Sutsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc. 1997;72:26–31. - PubMed
-
- Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990;82:507–513. - PubMed
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials
Miscellaneous