Atrial involvement in arrhythmogenic right ventricular cardiomyopathy patients referred for ventricular arrhythmias
Lingmin Wu1, Jingru Bao1, Erpeng Liang1
1Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, People's Republic of China.
Insights
Atrial involvement is common in arrhythmogenic right ventricular cardiomyopathy (ARVC). Tricuspid regurgitation and reduced ejection fraction increase atrial dilation risk, but genotype does not influence atrial changes in ARVC patients.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable heart muscle disease primarily affecting the ventricles.
- Atrial involvement in ARVC is not well understood, prompting this investigation into its prevalence and predictors.
Purpose of the Study:
- To assess the incidence and characteristics of atrial involvement in ARVC patients.
- To investigate the role of genotype in ARVC-related atrial disease.
- To identify predictors of atrial dilation and arrhythmias in ARVC.
Main Methods:
- Screening of nine known ARVC-causing genes in affected individuals.
- Assessment of atrial dilation (right and left atria) and atrial tachyarrhythmias (ATa).
- Correlation analysis between genotype, clinical factors (tricuspid regurgitation, LVEF), and atrial involvement.
Main Results:
- Atrial involvement, including right atrium (RA) dilation (45%) and left atrium (LA) dilation (16%), was frequent.
- Mutation carriers showed increased RA dilation (54.7%) compared to non-carriers (27.8%).
- Tricuspid regurgitation and decreased left ventricular ejection fraction (LVEF) were significant predictors of atrial dilation, while genotype did not show a significant association.
Conclusions:
- Atrial involvement is a common finding in ARVC.
- Clinical factors like tricuspid regurgitation and reduced LVEF, rather than genotype, are associated with atrial dilation.
- Atrial involvement did not significantly impact long-term survival in this ARVC cohort.
Background:
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardium disorder that predominantly affects the ventricle. Little is known about atrial involvement. This study aimed to assess atrial involvement, especially the role of genotype on atrium in ARVC.
Methods:
The incidence, characterization and predictors of atrial involvement were investigated. Nine known ARVC-causing genes were screened and the correlation between genotype and atrial involvement was assessed.
Results:
Right atrium (RA) dilation, left atrium (LA) dilation, and sustained atrial tachyarrhythmias (ATa) were found in 45, 16 and 3 patients, respectively. Gene mutations were identified in 64 (64.0%) patients. Mutation carriers showed more RA dilation than noncarriers (54.7% vs. 27.8%, P = 0.009), and no difference in LA dilation and ATa. Multivariate analysis showed tricuspid regurgitation (OR: 18.867; 95% CI: 1.466-250.000; P = 0.024) increased the risk of RA dilation and decreased left ventricular ejection fraction (LVEF) (OR: 1.134; 95% CI: 1.002-1.272; P = 0.031) correlated with LA dilation, whereas genotype showed no significant effect. At a median follow-up time of 91 months, 7 patients died and 1 patient accepted heart transplantation. New-onset RA dilation, LA dilation, and sustained ATa were found in 8, 7, and 6 patients, respectively. Atrial involvement was not associated with the long-term survival. Despite mutation carriers showing more RA dilation, Kaplan-Meier analysis showed genotype was not associated with atrial involvement.
Conclusion:
Atrial involvement was common in ARVC. Tricuspid regurgitation and decreased LVEF increased the risk for atrial dilation. Genotype was not associated with atrial involvement.
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