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Arrhythmogenic Right Ventricular Cardiomyopathy (arrhythmogenic + right_ventricular_cardiomyopathy)
Selected AbstractsChanges in the Isolated Delayed Component as an Endpoint of Catheter Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy: Predictor for Long-Term SuccessJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2008AKIHIKO NOGAMI M.D. Introduction: Although successful ablation of ventricular tachycardia (VT) is feasible in arrhythmogenic right ventricular cardiomyopathy (ARVC), long-term recurrence is common. The aim of this study was to assess the usefulness of a change in the isolated delayed component (IDC) as an endpoint of the catheter ablation in ARVC. Methods and Results: Eighteen patients (48 ± 11 years) with ARVC were studied. Detailed endocardial mapping of the right ventricle (RV) was performed during sinus rhythm. IDCs were recorded in 16 patients and the latest IDCs were related to the VT circuit. Catheter ablation was carried out in the areas with the IDCs. At the end of the session, the IDC was electrically dissociated in one, disappeared in five, exhibited second-degree block in one, was significantly delayed (,50 ms) in three, and remained unchanged in six. The change in the IDC was correlated with the change in the type II/III late potentials in the signal-averaged electrocardiography (ECG) and the inducibility of the clinical VT after the ablation. During a follow-up of 61 ± 38 months, VT recurred in six. The patients with a changed IDC had a significantly lower VT recurrence than those with no IDC or an unchanged IDC (P < 0.02). Conclusion: In patients with ARVC, (1) the IDCs during sinus rhythm are related to the clinical VT and can be a target for the ablation, (2) a change in the IDC can be used as an endpoint, and (3) qualitative analyses of the serial signal-averaged ECGs may be useful for the long-term follow-up. [source] Electrocardiographic Hallmark of Arrhythmogenic Right Ventricular CardiomyopathyJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 5 2006SERGIO RICHTER M.D. [source] Temporal Variability of Ventricular Arrhythmias in Boxer Dogs with Arrhythmogenic Right Ventricular CardiomyopathyJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 5 2009B.A. Scansen Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is prevalent in the Boxer. There is little information on the temporal variability of ventricular arrhythmias within affected dogs. Objective: To evaluate ambulatory electrocardiograms (AECG) from Boxers with ARVC for hourly variation in premature ventricular complexes (PVC) and heart rate (HR). Animals: One hundred and sixty-two Boxer dogs with ARVC. Methods: Retrospective, observational study of 1,181 AECGs collected from Boxer dogs at The Ohio State University from 1997 to 2004 was evaluated. The proportion of depolarizations that were PVCs was compared across each hour of the day, during six 4-hour periods of day, to the time after AECG application, and to the maximum and minimum HR. Results: A lower proportion of PVCs was noted during early morning (midnight to 0400 hours) as compared with the morning (0800,1200 hours) and late (1600,2000 hours) afternoon (P= .012). There was no increase in PVC proportion in the 1st hour after AECG application as compared with all other hours of the day (P= .06). There was poor correlation between maximum (,= 0.19) and minimum (,= 0.12) HR and PVC proportion. Conclusions and Clinical Importance: The likelihood of PVC occurrence in Boxer dogs with ARVC was relatively constant throughout the day, although slightly greater during the hours of 0800,1200 and 1600,2000. A biologically important correlation with HR was not apparent. The role of autonomic activity in the modulation of electrical instability in the Boxer with ARVC requires further study. [source] Signal-Averaged Electrocardiographic Parameter Progression as a Marker of Increased Electrical Instability in Two Cases with an Overt Form of Arrhythmogenic Right Ventricular CardiomyopathyPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 3 2002BARBARA BAUCE BAUCE, B., et al.: Signal-Averaged Electrocardiographic Parameter Progression as a Marker of In-creased Electrical Instability in Two Cases with an Overt Form of Arrhythmogenic Right Ventricular Cardiomyopathy. In arrhythmogenic right ventricular cardiomyopathy (ARVC) the fibrofatty substitution of the RV myocardium constitutes the substrate for reentrant circuits, leading to the onset of ventricular arrhythmias. This pathological process also accounts for "delayed ventricular potentials" that could be recorded as late potentials using the signal-averaged ECG technique (SAECG). This study examined two patients affected by overt forms of ARVC who showed a worsening of the electrical instability associated with a fast progression of SAECG parameters, while all the other clinical findings remained unchanged. This suggests a possible role of SAECG parameter progression as a marker of increased electrical instability. [source] Correlation between the Parameters of Signal-Averaged ECG and Two-Dimensional Echocardiography in Patients with Arrhythmogenic Right Ventricular CardiomyopathyANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 1 2009Yongwhi Park M.D. Background: The correlation between parameters of two-dimensional echocardiography and signal-averaged ECG (SAECG) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not known well. Methods: Thirty-three patients (13 females, 40.3 ± 14.4 years old) were included in this study. Both the right and left ventricular dimensions and systolic function were assessed with two-dimensional echocardiography. The SAECG was performed with high-gain amplification and filtered using bidirectional Butterworth filters between 40 and 250 Hz. We evaluated the correlation between the parameters of the SAECG and two-dimensional echocardiography. Results: The right ventricular (RV) outflow tract was the most frequently (n = 18, 54%) involved segment. Six (18%) patients had only mildly decreased RV systolic function. All the other patients had normal RV systolic function. Although localized left ventricular wall motion abnormalities were observed in 14 (42%) patients, the left ventricular ejection fraction was normal in most (n = 32, 97%). Late potentials were positive in 22 (63%) patients. There was no significant correlation between parameters of the SAECG and two-dimensional echocardiography for the entire patient population. Conclusions: The SAECG parameters exhibited no correlation to any of two-dimensional echocardiography parameters in the patients with ARVC. Fragmented electrical activity may develop with no significant relation to the anatomical changes in the patients with ARVC. [source] Arrhythmogenic Right Ventricular Cardiomyopathy: A Paradigm of Overlapping DisordersANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 4 2008Jeffrey A. Towbin M.D. No abstract is available for this article. [source] Arrhythmogenic Right Ventricular Dyspiasia/Cardiomyopathy:JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2000Need for an International Registry. Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right ventricular involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since its first description in 1977. This article will focus on the important hut still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed to answer the pending questions. The registry also will facilitate pathologic, molecular, and genetics research on the etiology and pathogenesis of the disease. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C. [source] Activation Delay and VT Parameters in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Toward Improvement of Diagnostic ECG CriteriaJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 8 2008MONIEK G.P.J. COX M.D. Introduction: Desmosomal changes, electrical uncoupling, and surviving myocardial bundles embedded in fibrofatty tissue are hallmarks of activation delay in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Currently, generally accepted task force criteria (TFC) are used for clinical diagnosis. We propose additional criteria based on activation delay and ventricular tachycardia (VT) to improve identification of affected individuals. Methods and Results: Activation delay and VT-related 12-lead electrocardiographic (ECG) criteria were studied, while off drugs, in 42 patients with proven ARVD/C according to TFC, and 27 controls with idiopathic VT from the RV outflow tract. Two of three measured TFC could only be identified in a small minority of ARVD/C patients. Additional ECG criteria proposed in this study included (a) prolonged terminal activation duration, an indicator of activation delay; (b) VT with LBBB morphology and superior axis; and (c) multiple different VT morphologies. These criteria were met in 30 (71%), 28 (67%), and 37 (88%) ARVD/C patients, respectively, and in one control patient (P < 0.001). Electrophysiologic studies contributed importantly to yield different VT morphologies. Pathogenic plakophilin-2 mutations were identified in 25 (60%) of ARVD/C patients and in none of the controls. In ARVD/C patients, parameters measured were not significantly different between mutation carriers and noncarriers, except for negative T waves in V1,3, occurring more frequently in patients with mutation. Conclusions: The proposed additional criteria are specific for ARVD/C and more sensitive than the current TFC. Therefore, adding the newly proposed criteria to current TFC could improve ARVD/C diagnosis, independent of DNA analysis. [source] Arrhythmogenic Right Ventricular Dyspiasia/Cardiomyopathy:JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2000Need for an International Registry. Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right ventricular involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since its first description in 1977. This article will focus on the important hut still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed to answer the pending questions. The registry also will facilitate pathologic, molecular, and genetics research on the etiology and pathogenesis of the disease. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C. [source] Temporal Variability of Ventricular Arrhythmias in Boxer Dogs with Arrhythmogenic Right Ventricular CardiomyopathyJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 5 2009B.A. Scansen Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is prevalent in the Boxer. There is little information on the temporal variability of ventricular arrhythmias within affected dogs. Objective: To evaluate ambulatory electrocardiograms (AECG) from Boxers with ARVC for hourly variation in premature ventricular complexes (PVC) and heart rate (HR). Animals: One hundred and sixty-two Boxer dogs with ARVC. Methods: Retrospective, observational study of 1,181 AECGs collected from Boxer dogs at The Ohio State University from 1997 to 2004 was evaluated. The proportion of depolarizations that were PVCs was compared across each hour of the day, during six 4-hour periods of day, to the time after AECG application, and to the maximum and minimum HR. Results: A lower proportion of PVCs was noted during early morning (midnight to 0400 hours) as compared with the morning (0800,1200 hours) and late (1600,2000 hours) afternoon (P= .012). There was no increase in PVC proportion in the 1st hour after AECG application as compared with all other hours of the day (P= .06). There was poor correlation between maximum (,= 0.19) and minimum (,= 0.12) HR and PVC proportion. Conclusions and Clinical Importance: The likelihood of PVC occurrence in Boxer dogs with ARVC was relatively constant throughout the day, although slightly greater during the hours of 0800,1200 and 1600,2000. A biologically important correlation with HR was not apparent. The role of autonomic activity in the modulation of electrical instability in the Boxer with ARVC requires further study. [source] Arrhythmogenic Right Ventricular Dyspiasia/Cardiomyopathy:JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2000Need for an International Registry. Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right ventricular involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since its first description in 1977. This article will focus on the important hut still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed to answer the pending questions. The registry also will facilitate pathologic, molecular, and genetics research on the etiology and pathogenesis of the disease. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C. [source] Changes in the Isolated Delayed Component as an Endpoint of Catheter Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy: Predictor for Long-Term SuccessJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 7 2008AKIHIKO NOGAMI M.D. Introduction: Although successful ablation of ventricular tachycardia (VT) is feasible in arrhythmogenic right ventricular cardiomyopathy (ARVC), long-term recurrence is common. The aim of this study was to assess the usefulness of a change in the isolated delayed component (IDC) as an endpoint of the catheter ablation in ARVC. Methods and Results: Eighteen patients (48 ± 11 years) with ARVC were studied. Detailed endocardial mapping of the right ventricle (RV) was performed during sinus rhythm. IDCs were recorded in 16 patients and the latest IDCs were related to the VT circuit. Catheter ablation was carried out in the areas with the IDCs. At the end of the session, the IDC was electrically dissociated in one, disappeared in five, exhibited second-degree block in one, was significantly delayed (,50 ms) in three, and remained unchanged in six. The change in the IDC was correlated with the change in the type II/III late potentials in the signal-averaged electrocardiography (ECG) and the inducibility of the clinical VT after the ablation. During a follow-up of 61 ± 38 months, VT recurred in six. The patients with a changed IDC had a significantly lower VT recurrence than those with no IDC or an unchanged IDC (P < 0.02). Conclusion: In patients with ARVC, (1) the IDCs during sinus rhythm are related to the clinical VT and can be a target for the ablation, (2) a change in the IDC can be used as an endpoint, and (3) qualitative analyses of the serial signal-averaged ECGs may be useful for the long-term follow-up. [source] Long-Term Follow-Up After Radiofrequency Catheter Ablation of Ventricular Tachycardia: A Successful Approach?JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 5 2002ALIDA E. BORGER VAN DER BURG M.D. RF Catheter Ablation of VT.Introduction: Radiofrequency ablation (RFCA) of ventricular tachycardia (VT) is a potential curative treatment modality. We evaluated the results of RFCA in patients with VT. Methods and Results: One hundred fifty-one consecutive patients (122 men and 29 women; age 57 ± 16 years) with drug-refractory VT were treated. Underlying heart disease was ischemic heart disease in 89 (59%), arrhythmogenic right ventricular cardiomyopathy (ARVC) in 32 (21%), and idiopathic VT in 30 (20%; left ventricle in 9 [30%]; right ventricle in 21 [70%]). Ablation was performed using standard ablation techniques. Three hundred six different VTs were treated (cycle length 334 ± 87 msec, 2.0 ± 1.4 VTs per patient). Procedural success (noninducibility of VT after RFCA) was achieved in 126 (83%) patients (70 ischemic heart disease [79%]; 28 ARVC [88%]; 27 idiopathic VT [93%]). Procedure-related complications (< 48 hours) occurred in 11 (7%) patients: death 3 (2.0%), cerebrovascular accident 2 (1.3%), complete heart block 4 (2.6%), and pericardial effusion 3 (2.0%). Thirty-three (22%) patients received an implantable cardioverter defibrillator (because of hemodynamic unstable VT, failure of the procedure, or aborted sudden death). During follow-up (34 ± 11 months), VT recurrences occurred in 38 (26%) patients (recurrence rate: 19% in successfully ablated patients and 64% in nonsuccessfully ablated patients; P < 0.001). During follow-up, 12 (8%) patients died (heart failure 8, unknown cause 1, noncardiac cause 3). Conclusion: RFCA of VT can be performed with a high degree of success (83%). The long-term outcome of successfully ablated patients is promising, with a 75% relative risk reduction compared with nonsuccessfully ablated patients. During follow-up, only one patient died suddenly, supporting a selective ICD placement approach in patients with hemodynamically stable VT. [source] Heart Rate Variability in Arrhythmogenic Right Ventricular Cardiomyopathy Correlation with Clinical and Prognostic FeaturesPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 9 2002ANTONIO FRANCO FOLINO FOLINO, A.F., et al.: Heart Rate Variability in Arrhythmogenic Right Ventricular Cardiomyopathy Correlation with Clinical and Prognostic Features. The identification of subjects with arrhythmogenic right ventricular cardiomyopathy (ARVC) at higher risk for sudden death is an unresolved issue. An influence of the autonomic activity on the genesis of ventricular arrhythmias was postulated. Heart rate variability (HRV) analysis provides a useful method to measure autonomic activity, and is a predictor of increased risk of death after myocardial infarction. For these reasons, the aim of the study was to evaluate HRV and its correlations with ventricular arrhythmias, heart function, and prognostic outcome in patients with ARVC. The study included 46 patients with ARVC who were not taking antiarrhythmic medications. The diagnosis was made by ECG, echocardiography, angiography, and endomyocardial biopsy. Exercise stress test and Holter monitoring were obtained in all patients. Time-domain analysis of HRV was expressed as the standard deviation of all normal to normal NN intervals (SDNN) detected during 24-hour Holter monitoring. Thirty healthy subjects represented a control group for HRV analysis. The mean follow-up was 10.8 ± 1.86 years. SDNN was reduced in patients with ARVC in comparison with the control group (151 ± 36 vs 176 ± 34, P = 0.00042). Moreover, there was a significant correlation of this index with the age of the patients (r =,0.59, P < 0.001), with the left (r = 0.44, P = 0.002) and right (r = 0.47, P = 0.001) ventricle ejection fraction, with the right ventricular end diastolic volume (r =,0.62, P < 0.001), and with the ventricular arrhythmias, detected during the same Holter record used for HRV analysis (patients with isolated ventricular ectopic beats < 1,000/24 hours, 184 ± 34; patients with isolated ventricular ectopic beats > 1,000/24 hours and/or couplets, 156 ± 25; patients with repetitive ventricular ectopic beats (,3) and/or ventricular tachycardia, 129 ± 25; P < 0.001). During follow-up two patients showed a transient but significant reduction of SDNN and a concomitant increase of the arrhythmic events. In eight patients an episode of sustained ventricular tachycardia occurred, but the mean SDNN of this subgroup did not differ from the mean value of the remaining patients (152 ± 15 vs 150 ± 39; P = NS). Only one subject died after heart transplantation during follow-up (case censored). Time-domain analysis of HRV seems to be a useful method to assess the autonomic influences in ARVC. A reduction of vagal influences correlates with the extent of the disease. The significant correlation between SDNN and ventricular arrhythmias confirmed the influences of autonomic activity in the modulation of the electrical instability in ARVC patients. However, SDNN was not predictive of spontaneous episodes of sustained ventricular tachycardia. [source] Signal-Averaged Electrocardiographic Parameter Progression as a Marker of Increased Electrical Instability in Two Cases with an Overt Form of Arrhythmogenic Right Ventricular CardiomyopathyPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 3 2002BARBARA BAUCE BAUCE, B., et al.: Signal-Averaged Electrocardiographic Parameter Progression as a Marker of In-creased Electrical Instability in Two Cases with an Overt Form of Arrhythmogenic Right Ventricular Cardiomyopathy. In arrhythmogenic right ventricular cardiomyopathy (ARVC) the fibrofatty substitution of the RV myocardium constitutes the substrate for reentrant circuits, leading to the onset of ventricular arrhythmias. This pathological process also accounts for "delayed ventricular potentials" that could be recorded as late potentials using the signal-averaged ECG technique (SAECG). This study examined two patients affected by overt forms of ARVC who showed a worsening of the electrical instability associated with a fast progression of SAECG parameters, while all the other clinical findings remained unchanged. This suggests a possible role of SAECG parameter progression as a marker of increased electrical instability. [source] Anaesthetic implications of arrhythmogenic right ventricular dysplasia/cardiomyopathyANAESTHESIA, Issue 1 2009A. K. Alexoudis Summary Arrhythmogenic right ventricular dysplasia, also called right ventricular cardiomyopathy, is a genetically determined heart muscle disease, characterised by life-threatening ventricular arrhythmias in apparently healthy young people. The primary myocardial pathology is that the myocardium of the right ventricular free wall is replaced by fibrous or fibrofatty tissue, with scattered residual myocardial cells. Right ventricular function is abnormal and in severe cases is associated with global right ventricular dilation and overt biventricular heart failure. Although still relatively rare, arrhythmogenic right ventricular cardiomyopathy is a well recognised cause of sudden unexpected peri-operative death. In this review, we describe the basic characteristics of this disease, emphasising the diagnosis and we offer some suggestions for the anaesthetic management of these patients in the peri-operative period. [source] Correlation between the Parameters of Signal-Averaged ECG and Two-Dimensional Echocardiography in Patients with Arrhythmogenic Right Ventricular CardiomyopathyANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 1 2009Yongwhi Park M.D. Background: The correlation between parameters of two-dimensional echocardiography and signal-averaged ECG (SAECG) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is not known well. Methods: Thirty-three patients (13 females, 40.3 ± 14.4 years old) were included in this study. Both the right and left ventricular dimensions and systolic function were assessed with two-dimensional echocardiography. The SAECG was performed with high-gain amplification and filtered using bidirectional Butterworth filters between 40 and 250 Hz. We evaluated the correlation between the parameters of the SAECG and two-dimensional echocardiography. Results: The right ventricular (RV) outflow tract was the most frequently (n = 18, 54%) involved segment. Six (18%) patients had only mildly decreased RV systolic function. All the other patients had normal RV systolic function. Although localized left ventricular wall motion abnormalities were observed in 14 (42%) patients, the left ventricular ejection fraction was normal in most (n = 32, 97%). Late potentials were positive in 22 (63%) patients. There was no significant correlation between parameters of the SAECG and two-dimensional echocardiography for the entire patient population. Conclusions: The SAECG parameters exhibited no correlation to any of two-dimensional echocardiography parameters in the patients with ARVC. Fragmented electrical activity may develop with no significant relation to the anatomical changes in the patients with ARVC. [source] Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular DysplasiaJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 5 2009SMIT C. VASAIWALA M.D. Introduction: Case studies indicate that cardiac sarcoid may mimic the clinical presentation of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, the incidence and clinical predictors to diagnose cardiac sarcoid in patients who meet International Task Force criteria for ARVD/C are unknown. Methods and Results: Patients referred for evaluation of left bundle branch block (LBBB)-type ventricular arrhythmia and suspected ARVD/C were prospectively evaluated by a standardized protocol including right ventricle (RV) cineangiography-guided myocardial biopsy. Sixteen patients had definite ARVD/C and four had probable ARVD/C. Three patients were found to have noncaseating granulomas on biopsy consistent with sarcoid. Age, systemic symptoms, findings on chest X-ray or magnetic resonance imaging (MRI), type of ventricular arrhythmia, RV function, ECG abnormalities, and the presence or duration of late potentials did not discriminate between sarcoid and ARVD/C. Left ventricular dysfunction (ejection fraction <50%) was present in 3/3 patients with cardiac sarcoid, but only 2/17 remaining patients with definite or probable ARVD/C (P = 0.01). Conclusions: In this prospective study of consecutive patients with suspected ARVD/C evaluated by a standard protocol including biopsy, the incidence of cardiac sarcoid was surprisingly high (15%). Clinical features, with the exception of left ventricular dysfunction and histological findings, did not discriminate between the two entities. [source] Activation Delay and VT Parameters in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Toward Improvement of Diagnostic ECG CriteriaJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 8 2008MONIEK G.P.J. COX M.D. Introduction: Desmosomal changes, electrical uncoupling, and surviving myocardial bundles embedded in fibrofatty tissue are hallmarks of activation delay in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Currently, generally accepted task force criteria (TFC) are used for clinical diagnosis. We propose additional criteria based on activation delay and ventricular tachycardia (VT) to improve identification of affected individuals. Methods and Results: Activation delay and VT-related 12-lead electrocardiographic (ECG) criteria were studied, while off drugs, in 42 patients with proven ARVD/C according to TFC, and 27 controls with idiopathic VT from the RV outflow tract. Two of three measured TFC could only be identified in a small minority of ARVD/C patients. Additional ECG criteria proposed in this study included (a) prolonged terminal activation duration, an indicator of activation delay; (b) VT with LBBB morphology and superior axis; and (c) multiple different VT morphologies. These criteria were met in 30 (71%), 28 (67%), and 37 (88%) ARVD/C patients, respectively, and in one control patient (P < 0.001). Electrophysiologic studies contributed importantly to yield different VT morphologies. Pathogenic plakophilin-2 mutations were identified in 25 (60%) of ARVD/C patients and in none of the controls. In ARVD/C patients, parameters measured were not significantly different between mutation carriers and noncarriers, except for negative T waves in V1,3, occurring more frequently in patients with mutation. Conclusions: The proposed additional criteria are specific for ARVD/C and more sensitive than the current TFC. Therefore, adding the newly proposed criteria to current TFC could improve ARVD/C diagnosis, independent of DNA analysis. [source] Anaesthetic implications of arrhythmogenic right ventricular dysplasia/cardiomyopathyANAESTHESIA, Issue 1 2009A. K. Alexoudis Summary Arrhythmogenic right ventricular dysplasia, also called right ventricular cardiomyopathy, is a genetically determined heart muscle disease, characterised by life-threatening ventricular arrhythmias in apparently healthy young people. The primary myocardial pathology is that the myocardium of the right ventricular free wall is replaced by fibrous or fibrofatty tissue, with scattered residual myocardial cells. Right ventricular function is abnormal and in severe cases is associated with global right ventricular dilation and overt biventricular heart failure. Although still relatively rare, arrhythmogenic right ventricular cardiomyopathy is a well recognised cause of sudden unexpected peri-operative death. In this review, we describe the basic characteristics of this disease, emphasising the diagnosis and we offer some suggestions for the anaesthetic management of these patients in the peri-operative period. [source] | ||||||||||