Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is considered a progressive cardiomyopathy. However, data on the clinical features of disease progression are limited. The aim of this study was to assess 12-lead surface electrocardiographic (ECG) changes during long-term follow-up, and to compare these findings with echocardiographic data in our large cohort of patients with ARVC/D. Baseline and follow-up ECGs of 111 patients from three tertiary care centers in Switzerland were systematically analyzed with digital calipers by two blinded observers, and correlated with findings from transthoracic echocardiography. The median follow-up was 4 years (IQR 1.9–9.2 years). ECG progression was significant for epsilon waves (baseline 14% vs. follow-up 31%, p = 0.01) and QRS duration (111 ms vs. 114 ms, p = 0.04). Six patients with repolarization abnormalities according to the 2010 Task Force Criteria at baseline did not display these criteria at follow-up, whereas in all patients with epsilon waves at baseline these depolarization abnormalities also remained at follow-up. T wave inversions in inferior leads were common (36% of patients at baseline), and were significantly associated with major repolarization abnormalities (p = 0.02), extensive echocardiographic right ventricular involvement (p = 0.04), T wave inversions in lateral precordial leads (p = 0.05), and definite ARVC/D (p = 0.05). Our data supports the concept that ARVC/D is generally progressive, which can be detected by 12-lead surface ECG. Repolarization abnormalities may disappear during the course of the disease. Furthermore, the presence of T wave inversions in inferior leads is common in ARVC/D.

Randomized trials have demonstrated that implantable cardioverter defibrillator (ICD) therapy may reduce the risk of death in patients with non-ischaemic cardiomyopathy (CMP). In this study, we aimed at determining the long-term benefit of ICD therapy among patients with dilated CMP (DCM) and among those with other non-ischaemic cardiac diseases (NICDs). We performed a single-centre longitudinal study to assess the outcomes of 176 patients with NICDs who were implanted with an ICD for primary or secondary prevention of cardiac death. The cumulative survival rate after 1, 2, 5, and 10 years was 91, 87, 78, and 65%, respectively. Mortality risk did not differ significantly between patients with DCM and those with other NICDs. Atrial fibrillation, recurrent ventricular arrhythmias requiring ICD therapy, and right ventricular pacing, but not delayed intrinsic ventricular conduction, were associated with higher risk. New York Heart Association (NYHA) functional class ≥III was an independent predictor of adverse outcome among patients with DCM [hazard ratio (HR) 5.27, P = 0.01], whereas reduced left ventricular function with ejection fraction <35% (HR 12.1, P < 0.001) and anti-arrhythmic drug use (HR 4.82, P = 0.03) were independent predictors among those with other NICDs. Renal insufficiency with estimated glomerular filtration rate <60 mL/min/1.73 m ² (HR 5.9, P < 0.001) was a strong independent predictor of mortality among all patients with NICD, irrespective of underlying cardiac condition. In ICD patients with DCM, higher NYHA functional class is associated with adverse outcomes. Impaired left ventricular function and anti-arrhythmic drug use predict higher mortality among patients with non-dilated, NICDs. Impaired renal function is a strong predictor of mortality in all patients with NICD.

Natriuretic peptides have been shown to be high in patients with myocardial ischemia. We sought to create a diagnostic score using clinical data, stress testing, and B-type natriuretic peptide (BNP) levels to improve noninvasive prediction of coronary artery disease (CAD). Patients with stable angina pectoris and normal systolic left ventricular function were eligible for this prospective cohort study. Patients with arrhythmias, valvular heart disease, impaired left ventricular function, or renal dysfunction were excluded. All patients underwent clinical evaluation, bicycle stress testing, BNP testing, and coronary angiography. Then a diagnostic risk score was derived that combined cardiovascular risk factors, results of exercise testing, and BNP measurements and added 1 point for the presence of each of these variables. Seventy-one patients (52 years of age, range 31 to 61; 46 men) were included in the study. Prevalence of CAD, defined by 50% narrowing of > or =1 coronary artery on coronary angiography, was 45%. For 0 point in the risk score system, the negative predictive value was 93% with a negative likelihood ratio of 0.1 (95% confidence interval [CI] 0.02 to 0.38); for a score of 3 points, the positive predictive value was 93% with a positive likelihood ratio of 15.9 (95% CI 2.19 to 114.7). Serum BNP level >50 ng/L at rest was the best single diagnostic parameter, with 66% sensitivity and 97% specificity, and a positive likelihood ratio of 25.6 (95% CI 3.64 to 180) and a negative likelihood ratio of 0.35 (95% CI 0.22 to 0.57). In conclusion, a diagnostic score combining exercise testing, clinical data, and serum BNP values at rest can distinguish patients with CAD from those without CAD with high accuracy.