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- Title
Influence of Genotype on Structural Atrial Abnormalities and Atrial Fibrillation or Flutter in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy.
- Authors
BOURFISS, MIMOUNT; TE RIELE, ANNELINE S.J.M.; MAST, THOMAS P.; CRAMER, MAARTEN J.; HEIJDEN, JEROEN F.; VAN VEEN, TOON A.B.; LOH, PETER; DOOIJES, DENNIS; HAUER, RICHARD N.W.; VELTHUIS, BIRGITTA K.
- Abstract
Structural Atrial Involvement in ARVD/C Introduction Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is associated with desmosomal mutations. Although desmosomal disruption affects both ventricles and atria, little is known about atrial involvement in ARVD/C. Objective To describe the extent and clinical significance of structural atrial involvement and atrial arrhythmias (AA) in ARVD/C stratified by genotype. Methods We included 71 patients who met ARVD/C Task Force Criteria and underwent cardiac magnetic resonance (CMR) imaging and molecular genetic analysis. Indexed atrial end-diastolic volume and area-length-ejection-fraction (ALEF) were evaluated on CMR and compared to controls with idiopathic right ventricular outflow tract tachycardia (n = 40). The primary outcome was occurrence of AA (atrial fibrillation or atrial flutter) during follow-up, recorded by 12-lead ECG, Holter monitoring or implantable cardioverter defibrillator (ICD) interrogation. Results Patients harbored a desmosomal plakophilin-2 ( PKP2) (n = 37) or nondesmosomal phospholamban ( PLN) (n = 14) mutation. In 20 subjects, no pathogenic mutation was identified. Compared to controls, right atrial (RA) volumes were reduced in PKP2 (P = 0.002) and comparable in PLN (P = 0.441) mutation carriers. In patients with no mutation identified, RA (P = 0.011) and left atrial (P = 0.034) volumes were increased. Bi-atrial ALEF showed no significant difference between the groups. AA were experienced by 27% of patients and occurred equally among PKP2 (30%) and no mutation identified patients (30%), but less among PLN mutation carriers (14%). Conclusion Genotype influences atrial volume and occurrence of AA in ARVD/C. While the incidence of AA is similar in PKP2 mutation carriers and patients with no mutation identified, PKP2 mutation carriers have significantly smaller atria. This suggests a different arrhythmogenic mechanism.
- Subjects
ATRIAL arrhythmias; ATRIAL fibrillation; CHI-squared test; ELECTROCARDIOGRAPHY; FISHER exact test; HEART atrium; MAGNETIC resonance imaging; CARDIOMYOPATHIES; T-test (Statistics); ATRIAL flutter; DATA analysis software; MANN Whitney U Test; ARRHYTHMOGENIC right ventricular dysplasia; GENOTYPES; KRUSKAL-Wallis Test; ONE-way analysis of variance
- Publication
Journal of Cardiovascular Electrophysiology, 2016, Vol 27, Issue 12, p1420
- ISSN
1045-3873
- Publication type
Article
- DOI
10.1111/jce.13094