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CONTEXT QRS fragmentation, including epsilon potentials, terminal activation delay and prolonged S wave upstroke, has been recently described as a diagnostic criterion of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Whether QRS fragmentation is a marker of recurrent ventricular tachycardia, primary ventricular fibrillation, implantable cardioverter defibrillator (ICD) discharge and sudden cardiac death in these patients is unknown. RESULTS Three hundred and thirty-five patients (167 men, mean age 46.3 ± 14.6 years) with ARVC/D according to International Society and Federation of Cardiology/European Society of Cardiology (ISFC/ESC) criteria were analyzed retrospectively. Patients with complete and incomplete right bundle branch block were excluded from the analysis. At 6.3 ± 3.1 years mean follow-up, seven patients (0.02%) had died suddenly, 39 patients (0.13%) experienced recurrent ventricular tachycardia, 32 patients (0.1%) presented with primary ventricular fibrillation and 30 patients (0.1%) had recurrent ICD discharges. QRS fragmentation was significantly associated with arrhythmic events (P

Higher doses of oral valsartan (160-320 mg) seem to reduce in-stent restenosis rate after bare-metal stent implantation. The value of 80, 160 or 320 mg valsartan should be analyzed by late lumen loss and follow-up percent diameter stenosis as surrogate parameters in a total of 60 patients with matched demographic, clinical and angiographic findings and continuous doses of valsartan. In each group 20 patients (14 males, 6 females) with a mean age of 62.1+/-9.1, 64.3+/-8.1 and 62.9+/-11.6 years after implantation of a total of 22, 33 and 27 stents in 21, 25 and 23 lesions were included. Quantitative coronary angiography was performed with an automated contour analysis system; reference diameter, minimum diameter, late lumen loss, follow-up percent diameter stenosis and restenosis rate were determined.In-stent restenosis rate including persistent area was n=5/21 (24%), n=4/25 (16%) and n=2/23 (8.7%) under 80, 160 and 320 mg valsartan. Late lumen loss was 0.79+/-0.49 mm, 0.60+/-0.43 mm and 0.37+/-0.25 mm, respectively, with significant differences between 80 and 320 mg (p0.001) and 160 and 320 mg (p0.05). Follow-up percent diameter stenosis was 31.8+/-18.6% under 80 mg, 25.2+/-17.5% under 160 mg and 13.8+/-9.4% with significant differences between 80 mg and 320 mg (p0.0005) and 160 and 320 mg (p0.01).Different doses of oral valsartan over six months after BMS implantation show a linear response with regard to late lumen loss and follow-up percent diameter stenosis.

The use of ACE inhibitors (ACE-i) represents an Ia recommendation in the treatment of patients with STEMI and NSTEMI. However, results of smaller studies suggest an increase of in-stent-restenosis under ACE-i administration. The effects of ACE-i and valsartan after bare metal stent implantation of the culprit type B2/C lesion should be compared. Seven hundred patients were treated either by ACE-i in cases of LVEF50% or 80 mg valsartan in cases of LVEFor =50%. Restenosis rates after 6 months were analysed in 399 patients under valsartan and 224 patients under ACE-i with control angiography and major adverse cardiac events (death, infarction, reintervention) in a follow-up of up to 4 (mean 2.6) years in all patients. In-stent-restenosis was found in 19.5% under valsartan and in 34% under ACE-i (p0.005). In diabetic patients, restenosis occurred in 24% under valsartan and in 43% under ACE-i (p0.01). In initial acute coronary syndrome (ACS), restenosis rate was 14% under valsartan and 43% under ACE-i (p0.0001). In stable angina, restenosis rates were 26.5% and 27.5%, respectively. Total MACE rates revealed significant differences in ACS due to reintervention rates of 22% and 7% under ACE-i and valsartan (p0.0001). The administration of 80 mg valsartan after bare metal stent implantation leads to a reduction of in-stent-restenosis compared to ACE-i. This effect is mainly due to beneficial effects of valsartan in cases with initial ACS. Major differences between ACE-i and valsartan are discussed including inflammation, activation of neutrophils, mode of bradykinin activation, AT2 receptor stimulation and apoptosis of smooth muscle cells.

We searched for special features in patients with complete and incomplete right bundle branch block diagnosed as having arrhythmogenic right ventricular cardiomyopathy/dysplasia. Whether right bundle branch block is a frequent finding in arrhythmogenic right ventricular cardiomyopathy should be studied. The question is whether special features exist such as T-wave inversions, localized right precordial QRS prolongation and r'/s ratio1.ARVC could be diagnosed according to ISFC/ESC criteria in 374 patients. CRBBB was found in 22 cases (6%) and iCRBBB was present in 47 cases (12.5%). In CRBBB T wave inversions ≥ V4 was found in 10 cases (n.s.) and r'/s ratio1 was present in 12 cases (p0.001). In iCRBBB T wave inversions ≥ V4 was found in 10 cases (n.s.) and ST segment elevation in right precordial leads was present in 19 cases (p0.005). In all patients with ARVC localized right precordial QRS prolongation was found. Patients with CRBBB have a bad prognosis: 17 of 22 patients developed biventricular heart failure requiring heart transplantation and diuretic therapy.CRBBB and iCRBBB are infrequent findings in arrhythmogenic right ventricular cardiomyopathy. Complete right bundle branch block is characterized by r'/s ratio1. There are no significant T wave inversions ≥ V4. Incomplete right bundle branch block is characterized by ST segment elevation in right precordial leads but not by T wave inversions ≥ V4.

Background Epsilon potentials in right precordial leads are reliable diagnostic electrocardiographic (ECG) criteria of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C). Sensitivity of epsilon potentials can be enhanced by highly amplified and modified ECG recording technique. Nevertheless, in many cases the definition of epsilon potentials remains difficult. Objective To overcome these limitations, the value of QRS fragmentation in a standard 12-lead ECG was analyzed in 360 patients with ARVD/C (176 men, mean age 47.3 ± 13.7 years). Methods Analysis of QRS fragmentation of the whole collective of patients was compared with the detection of epsilon potentials in highly amplified right precordial and modified limb leads in a subgroup of 207 patients. Fifty-two phenotypically and genotypically nonaffected subjects from systematic family screening in 10 families with known plakophilin-2 and desmoplakin mutations served as a control group. Results QRS fragmentation could be found in a total of 306 of 360 patients (85%); 2.09 ± 1.8 fragmented QRS complexes (range 1 to 7) could be found per patient. Fragmented QRS complexes in only 1 right precordial lead were found in 106 cases. In 190 cases, QRS fragmentation was present in more than 1 lead, including all 12 standard leads. Epsilon potentials in highly amplified right precordial and modified limb leads could be found in a total of 159 cases (77%). Typical epsilon potentials in highly amplified right precordial leads could be found in 97 cases (47%). Conclusion QRS fragmentation in ARVD/C has a high diagnostic value similar to epsilon potentials by a highly amplified and modified recording technique.

Background The use of electrocardiographic (ECG) depolarization and repolarization criteria plays a large role in the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Different ECG algorithms should be analyzed in making the diagnosis of ARVD/C with the use of normal and modified recording techniques. Methods In a cohort of 343 patients (210 men and 133 women; mean age, 46.0 ± 13.7 years) meeting the Task Force of the Working Group on Myocardial and Pericardial Diseases of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology diagnostic criteria for ARVD/C, the value of different ECG criteria (eg, localized right precordial QRS prolongation defined as QRS duration in (V1+V2+V3)/(V4+V5+V6) of 1.2 or higher, right precordial QRS prolongation with QRS in V1-3 of 110 milliseconds or higher, epsilon potentials in the right precordial leads, S-wave upstroke in V1-3 of 55 milliseconds or higher, and right precordial T-wave inversions) was analyzed with the use of a normal recording technique and a highly amplified and modified recording technique (n = 207) at a paper speed of 50 mm/s. Fifty-two phenotypically and genotypically unaffected individuals identified by systematic screening in 24 families (30 men; mean age, 42.4 ± 8.3 years) were treated as control subjects. Results In the normal as well as highly amplified and modified recording techniques, the incidence of localized right precordial QRS prolongation was 98% (100%), that of QRS in V1-3 of 110 milliseconds or higher was 75% (80%), that of prolonged right precordial S-wave upstroke was 84% (60%), that of epsilon potentials was 23% (77%), and that of right precordial T-wave inversions was 55%. Four of 6 patients without the phenomenon of localized right precordial QRS prolongation with the use of the normal recording technique had a prolonged S-wave upstroke of 55 milliseconds or higher. In the control group, localized right precordial QRS prolongation, QRS in V1-3 of 110 milliseconds or higher, and epsilon potentials could not be identified. An S-wave upstroke of 55 milliseconds or higher was present in 2 of 3 cases, and T-wave inversions were found in 3. Conclusions Electrocardiographic depolarization criteria for ARVD/C analyzed in this large cohort of patients meeting the International Society and Federation of Cardiology/European Society of Cardiology criteria presented with high sensitivity and specificity in comparison with those in the control group of phenotypically and genotypically unaffected individuals defined by systematic screening in 24 families with ARVD/C. The incidence of right precordial T-wave inversions was much lower, indicating that not only patients with overt right ventricular dilatation and dysfunction were included. Electrocardiographic algorithms, including localized right precordial QRS prolongation, prolonged S-wave upstroke, and epsilon potentials, with the use of the normal recording technique and the amplified and modified recording technique at a paper speed of 50 mm/s contribute significantly to the noninvasive diagnosis of ARVD/C.

Syncopes appear in 10-20% in arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C). In the majority of cases sustained or non-sustained monomorphic ventricular tachycardias represent the underlying mechanism of syncope. In other cases the mechanism remains unclear. In 37 patients (23 females, mean age 43.6+/-12.8 years) without detectable and inducible monomorphic ventricular tachycardia, a diagnostic algorithm including repeat ECG, holter monitoring, telemetry, electrophysiological examination, ajmaline challenge, tilt table testing and neurological work-up (EEG, cranial computer tomography) was used in order to identify the mechanism of syncopes. Constant AV block 3 degrees could be found in 3 patients (2 males). Intermittant AV block 2 degrees or 3 degrees could be identified in 3 females. Four males had abnormal Wenckebach point during rapid atrial stimulation, 3 males demonstrate isolated HV interval prolongation. Rapid polymorphic VT and VF could be induced in a young female with ARVD/C. Eight patients (7 females) presented with recurrent syncopes and provocable right precordial ST elevation and right bundle branch block during ajmaline challenge. Three patients had abnormal tilt table testing as the only pathological finding. In one female with intermittent AV block 2 degrees tilt table testing and ajmaline challenge was positive. One female had the diagnosis of focal epilepsia after neurological work-up. In 11 cases the mechanism of syncopes remained unclear. In patients with ARVD/C and syncopes beyond detectable or inducible monomorphic VT, several mechanisms of syncopes could be identified with conduction disease as the predominant finding. These results may help in identifying rare mechanisms of syncopes in ARVD/C.

An association between arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) and Brugada syndrome can be supposed according to several case reports. In order to examine a possible link between ARVD/C and Brugada syndrome, systematic ajmaline testing with 1 mg/kg body weight intravenously, was done in 55 patients (32 males, mean age 46.7+/-12.3 years) with ISFC/ESC criteria of ARVD/C. In nine patients ajmaline testing could demonstrate coved ST segment elevation of at least 2 mm in at least two right precordial leads. Three of these patients had recurrent syncopes. Electrophysiological study revealed non-sustained ventricular tachycardia with left bundle branch block configuration and inferior axis in only one case. Systematic ajmaline testing could demonstrate a definite link between ARVD/C and Brugada syndrome.