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Background: Prior studies of structural and electrocardiographic changes in arrhythmogenic right ventricular (RV) cardiomyopathy and their role in predicting ventricular arrhythmias (ventricular tachycardia) have shown conflicting results. Methods: We reviewed 405 ECGs, 315 transthoracic echocardiographies, and 441 implantable cardioverter defibrillator interrogations in 64 arrhythmogenic RV cardiomyopathy patients (56% men, mean age [SD], 44.2 [14.6] years) over a mean follow-up of 10 (range, 2.3–19) years. Generalized estimating equations were used to identify the association between ECG abnormalities, clinical variables, and transthoracic echocardiographic measurements (&gt;mild degree of tricuspid regurgitation, RV outflow tract diameter in parasternal long axis and short axis, RV end-diastolic area, fractional area change). Results: There was a 4.65 (95% CI, 0.51%–8.8%) increase in RV end-diastolic area, a 3.75 (95% CI, 1.17%–6.34%) decrease in fractional area change, and 1.9 (95% CI, 1.3–2.8) higher odds (odds ratio) of RV wall motion abnormality with every 5-year increase in age after patients’ first transthoracic echocardiography. &gt;Mild tricuspid regurgitation was an independent predictor of RV enlargement and dysfunction (hazard ratio of &gt;10% drop in fractional area change from baseline [95% CI], 3.51 [1.77–6.95] and hazard ratio of &gt;10% increase in RV end-diastolic area from baseline [95% CI], 4.90 [2.52–9.52]). Patients with implantable cardioverter defibrillator were more likely to develop &gt;mild tricuspid regurgitation and larger structural and functional disease progression. More pronounced increase in RV end-diastolic area was translated into higher rates of any ventricular tachycardia. Inferior T-wave inversions and sum of R waves (mm) in V1 to V3 were predictors of RV enlargement and dysfunction with the former also predicting risk of any ventricular tachycardia. Conclusions: Arrhythmogenic RV cardiomyopathy is a progressive disease. Tricuspid regurgitation is an independent predictor of structural disease progression, which may be exacerbated by use of a transvenous implantable cardioverter defibrillator lead.

circulation. heart failure

Long-Term Electrocardiographic and Echocardiographic Progression of Arrhythmogenic Right Ventricular Cardiomyopathy and Their Correlation With Ventricular Tachyarrhythmias