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Right Ventricular Dysplasia (right + ventricular_dysplasia)

Distribution by Scientific Domains

Medical Sciences	100%

Kinds of Right Ventricular Dysplasia

arrhythmogenic right ventricular dysplasia

Selected Abstracts

Diagnostic Value of Endomyocardial Biopsy Guided by Electroanatomic Voltage Mapping in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 11 2008
ANDREA AVELLA M.D.
Introduction: To improve the endomyocardial biopsy (EMB) diagnostic sensitivity for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), we hypothesized a biopsy sampling focused on selected right ventricle (RV) low-voltage areas identified by electroanatomic voltage mapping. Methods and Results: The study population (22 patients, 10 men; mean age 34 ± 10 years) included 11 patients with overt ARVC/D (group A) and 11 patients with suspected ARVC/D (group B), according to both arrhythmic profile and standardized noninvasive diagnostic criteria. In all 22 patients, an RV bipolar voltage mapping was performed with CARTOÔ system sampling multiple endocardial sites (262 ± 61), during sinus rhythm, with a 0.5,1.5 mV color range setting of voltage display. All 11 (100%) group A patients and 8 of the 11 (73%) group B patients (P = nonsignificant [NS]) presented RV low-voltage areas (<0.5 mV). In 8 group A patients and in all 8 group B patients with a pathological RV voltage map, an EMB focused on the low-voltage areas was performed. In 6 (75%) group A patients and in 7 (87%) group B patients (P = NS), voltage mapping-guided EMB was diagnostic for ARVC/D. In the remaining 3 patients, only nonspecific histological findings were observed. Conclusions: The results of our study (1) confirm the high diagnostic sensitivity of RV voltage mapping in patients with overt ARVC/D, (2) document a high prevalence of RV low-voltage areas even in patients with suspected ARVC/D, and (3) demonstrate that in patients with clinical evidence or suspicion for ARVC/D, presenting RV low-voltage areas, EMB guided by voltage mapping may provide ARVC/D diagnosis confirmation. [source]

Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 5 2009
SMIT 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]

Pacing in Right Ventricular Dysplasia after Disconnection Surgery

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 2 2000
CHUEN TANG M.B.B.S.
Pacing in Right Ventricular Dysplasia. This report describes a 33-year-old patient with arrhythmogenic right ventricular (RV) dysplasia who had a dual chamber pacemaker implanted at age 23 years for drug-induced bradycardia. Pacing was continued after right ventricular free-wall disconnection (RVFVVD) at age 24 years. Her pacemaker was not replaced after battery depletion 7 years later. She presented the following year in severe right-sided heart failure. Her old pacemaker generator was replaced. This was followed by rapid resolution of her clinical failure and return to a full, active, physical lifestyle. This observation suggests the potential benefit of dual chamber pacing in patients with RV dysplasia after RVFWD. [source]

Patients with Scar-Related Right Ventricular Tachycardia: Determinants of Long-Term Outcome

JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 10 2009
ADRIANUS P. WIJNMAALEN M.D.
Introduction: Patients with established arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) based on task force (TF) criteria and ventricular tachycardia (VT) are at risk of VT recurrence and sudden death. Data on patients with VT due to right ventricular (RV) scar not fulfilling TF criteria are lacking. The purpose of this study was to assess the long-term arrhythmia recurrence rate and outcome in patients with scar-related right VT with and without a diagnosis of ARVC/D. Methods: Sixty-four patients (age 43.5 ± 15 years, 49 males) presenting with nonischemic scar-related VT of RV origin were studied. Scar was identified by electroanatomical mapping, contrast echocardiography, and/or magnetic resonance imaging (MRI). Patients were evaluated and treated according to a standard institute protocol. Results: Twenty-nine (45%) patients were diagnosed with ARVC/D according to TF criteria (TF+) and 35 (55%) with RV scar of undetermined origin (TF,) at the end of follow-up (64 ± 42 months). Patients were treated with antiarrhythmic drugs, radiofrequency catheter ablation, and/or implantable cardioverter-defibrillator (ICD) implantation. VT recurrence-free survival for TF+ and TF, was 76% versus 74% at 1 year and 45% versus 50% at 4 years (P = ns). Patients with fast index VT (cycle length [CL], 250 ms, n = 31) were more likely to experience a fast VT during follow-up than patients with a slow index VT (CL > 250 ms, n = 33) (61% vs 3%, P < 0.001). Conclusions: Scar-related RV VTs have a high recurrence rate in TF+ and TF, patients. Patients presenting with a fast index VT are at high risk for fast VT recurrence and may benefit most from ICD therapy. [source]

Diagnostic Value of Endomyocardial Biopsy Guided by Electroanatomic Voltage Mapping in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Characterization of the Electroanatomic Substrate for Monomorphic Ventricular Tachycardia in Patients with Nonischemic Cardiomyopathy

PACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 7 2002
HENRY H. HSIA
HSIA, H.H., et al.: Characterization of the Electroanatomic Substrate for Monomorphic Ventricular Tachycardia in Patients with Nonischemic Cardiomyopathy. Ventricular arrhythmias are common in the setting of nonischemic cardiomyopathy. The etiology for the cardiomyopathy is frequently not identified and the label of "idiopathic" is applied. Interstitial fibrosis with conduction system involvement and associated left bundle branch block characterizes the disease process in some patients and the mechanism for monomorphic ventricular tachycardia is commonly bundle branch reentry. However, most patients with nonischemic cardiomyopathy have VT due to myocardial reentry and demonstrate marked myocardial fibrosis and electrogram abnormalities. Although patient specific, the overall distribution of electroanatomic abnormalities appears to be equal on the endocardium and epicardium. The extent of electrogram abnormalities appears to parallel arrhythmia presentation and/or inducibility. Patients with sustained uniform morphology VT have the most extensive endocardial and epicardial electrogram abnormalities. Magnetic electroanatomic voltage mapping provides a powerful tool to characterize the location and extent of the arrhythmia substrate. Basal left ventricular myocardial involvement, as indexed by the location of contiguous electrogram abnormalities, is common in patients with sustained VT and left ventricular cardiomyopathy. The relatively equal distribution of electrogram abnormalities on the endocardium and epicardium, and the results of mapping and ablation attempts, suggest that critical parts of the reentrant circuit may be epicardial. Unique features of the electroanatomic substrate associated with cardiomyopathy due to Chagas' disease, sarcoidosis, and arrhythmogenic right ventricular dysplasia are also discussed. [source]

Anaesthetic implications of arrhythmogenic right ventricular dysplasia/cardiomyopathy

ANAESTHESIA, Issue 1 2009
A. 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]

Web-Based Virtual Cardiac Symposia: A New Approach for Worldwide Professional Medical Education

ANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 2 2007
Sergio J. Dubner M.D.
Aim: The Internet is an extremely powerful tool for the transmission of data and knowledge, and the question is whether this technology can be used effectively in continuing medical education. We present our experience with worldwide, web-based virtual symposia for practicing physicians. Methods: The International Society for Holter and Noninvasive Electrocardiography (ISHNE) decided four years ago to conduct a series of cardiology-related educational activities for physicians utilizing a web-based approach. Six educational events under the format of virtual symposia were held on the Internet during the years 2002 to 2006. These Internet events included symposia on Brugada syndrome (2002), the long QT syndrome (2004), arrhythmogenic right ventricular dysplasia (2005), atrial fibrillation (2005), heart failure (2006), and sudden cardiac death (2006). Results: During the past four years, there has been a dramatic and progressive increase in the number of physician registrants, the number of countries represented, and the number of lectures downloaded with each subsequent virtual symposium. For example, during the month of October 2006, the Internet-based sudden cardiac death symposium involved 14,087 physician registrants from 120 countries with 64,939 lectures downloaded. The top lecture was downloaded 11,251 times, and over 200 e-mail questions and replies were exchanged. The average time per visit to the web site was 12.5 minutes. Conclusion: The progressively increasing numbers of physician registrants from around the world who participated in these web-based, virtual symposia suggest that this approach is answering an unmet professional educational need. This Internet approach adds an important, new, low-cost dimension to continuing medical education. [source]