Special features of right bundle branch block in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.

UNLABELLED: 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 ratio<1. RESULTS: 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 ratio<1 was present in 12 cases (p<0.001). 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 (p<0.005). 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. CONCLUSIONS: CRBBB and iCRBBB are infrequent findings in arrhythmogenic right ventricular cardiomyopathy. Complete right bundle branch block is characterized by r'/s ratio<1. 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.

Late lumen loss and follow-up percent diameter stenosis at different doses of oral valsartan six months after bare-metal stent implantation in type B2/C coronary lesions.

UNLABELLED: 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. RESULTS: 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 (p<0.001) and 160 and 320 mg (p<0.05). 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 (p<0.0005) and 160 and 320 mg (p<0.01). CONCLUSIONS: 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.

QRS fragmentation in standard ECG as a diagnostic marker of arrhythmogenic right ventricular dysplasia-cardiomyopathy.

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.

The value of different electrocardiographic depolarization criteria in the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

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.

Mechanisms of syncopes in arrhythmogenic right ventricular dysplasia-cardiomyopathy beyond monomorphic ventricular tachycardia.

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.

Valsartan versus ACE inhibition after bare metal stent implantation--results of the VALVACE trial.

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 LVEF<50% or 80 mg valsartan in cases of LVEF> or =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 (p<0.005). In diabetic patients, restenosis occurred in 24% under valsartan and in 43% under ACE-i (p<0.01). In initial acute coronary syndrome (ACS), restenosis rate was 14% under valsartan and 43% under ACE-i (p<0.0001). 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 (p<0.0001). 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.

Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital.

In a cardiological department of a non-referral hospital responsible for 80,000 inhabitants with 2500 in-hospital patients and 1500 out-hospital patients per year, the prevalence, symptoms and prognosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) were examined retrospectively. From 1997 to 2002, ARVD/C was diagnosed in 35 females and 45 males (overall prevalence 1 in 1000 inhabitants) with a mean age of 45.6 years. Symptoms were chest pain (80%), palpitations (60%) and syncopes (30%), and clinical findings were repetitive ventricular premature beats (50%), supraventricular arrhythmias (30%), ventricular tachycardia (20%), aborted sudden death due to ventricular fibrillation (1%), right heart failure (4%), biventricular heart failure (1%) and high grade AV nodal block (4%). Endomyocardial biopsies were not performed. Aborted sudden death occurred in only one patient (0.3%) before the diagnosis was made, annual heart failure rate was 1%. No deaths appeared in a follow-up of 1-5 (mean 2.4) years with clinical assessment as the basis of diagnosis. The prevalence of ARVD/C is much higher and the prognosis better than expected from results of reference centers.

Results of ajmaline testing in patients with arrhythmogenic right ventricular dysplasia-cardiomyopathy.

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.

Diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy: value of standard ECG revisited.

BACKGROUND: The diagnostic dilemma in arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) is that a single diagnostic test does not exist and that there is a need for broadening diagnostic criteria. As standard ECG contributes significantly to clinical diagnosis and represents a tool for screening in family studies ECG data should be revisited. METHODS AND RESULTS: In a cohort of 265 patients (159 males, mean age 46.8 years) with ISFC/ESC criteria of ARVD/C ECG features were reevaluated. QRS duration in (V1 + V2 + V3)/(V4 + V5 + V6) > or = 1.2-called localized right precordial QRS prolongation-was present in 261/265 patients (98%) and represents the essential finding. Right precordial epsilon potentials were found in 23% in standard and in 75% in highly amplified and modified recording technique. Right precordial T wave inversions were present in 143 cases (54%) and ST-segment elevation of different types in 66 patients (25%). Localized prolongation of inferior QRS complexes could be found in 58 cases (22%), complete right bundle branch block with T inversions beyond V2 in most cases in 17 patients (6%), incomplete right bundle branch block in 38 cases (14%), pseudo-incomplete right bundle branch block in 8 patients (3%), and right precordial R wave reduction in 14 cases (5%). CONCLUSION: With regard to sensitivity and already known specificity an ECG score for the diagnosis of ARVD/C was developed with high probability of ARVD/C in cases with > or =4 points, possibly without the need for an additional imaging technique. Standard ECG with additional highly amplified and modified recording technique represents a single diagnostic test with high value in the clinical diagnosis of ARVD/C and should be used as a first line tool in noninvasive family screening.

Value of intracardiac ultrasound in the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy.

The value of imaging techniques such as transthoracic echocardiography, angiography and magnetic resonance imaging in the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD) is limited. First experiences with intracardiac ultrasound have been made during electrophysiological interventions. The ability of using intracardiac ultrasound in ARVD should be tested. In 25 patients with IFSC/ESC criteria of ARVD (nine males, 16 females) with a mean age of 54 (29-78) years suffering from sustained ventricular tachycardia in three cases, positive family history in four cases and syncopes in six cases intracardiac ultrasound was done using 6 French (Fr) 12.5 MHz catheters and the CLEAR VIEW ULTRA Intravascular System (Boston Scientific). Images were taken from the right ventricular apex, outflow tract and infundibulum. Results were compared to selective right ventricular angiography. Right ventricular (RV) angiography revealed bulges and a partial or complete loss of trabecular structure in 22 cases at the apex, in 13 cases at the infundibulum and in 14 cases at the right ventricular outflow tract. Intracardiac echocardiography was able to demonstrate sacculations in all patients at the apex, in 20 cases at the infundibulum and in 16 patients at the right ventricular outflow tract. Sacculations in all segments of the right ventricle were based on a partial or complete loss of trabecular structure. A whole of 36 segments presented with wall thinning (<3 mm) and 15 segments with normal wall structure and wall thickening of surrounding myocardium (>4 mm). In 26 segments the structure of right ventricular wall was inhomogeneous. In comparison to angiography as the 'gold standard' intracardiac ultrasound presented with additional details in 12 cases and the demonstration of angiographic misinterpretation in one case. Intracardiac ultrasound in ARVD is feasible in all cases with 6 Fr 12.5 MHz catheters and provides additional information to the angiographic phenomenon of bulges and to the aspect of tissue characterisation.