Abstract 1768: Magnetic Resonance Imaging Reveals Novel Phenotypic Expression of Desmosomal Mutation in First-Degree Relatives of Patients with Arrhythmogenic Right Ventricular Dysplasia (ARVD). Prevalence and Clinical Significance in Patients Evaluated For ARVD.

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Harikrishna Tandri ◽  
Darshan Dalal ◽  
Aditya Jain ◽  
Daniel P Judge ◽  
Theodore Abraham ◽  
...  
Keyword(s):  
Family History ◽  
Right Ventricular ◽  
Phenotypic Expression ◽  
Clinical History ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
First Degree Relatives ◽  
Group 4 ◽  
Right Ventricular Dysplasia ◽  
History Of ◽  
The Right

Background: In this report we present the prevalence and significance of a novel MRI abnormality that we identified in desmosomal mutation positive asymptomatic relatives of arrhythmogenic right ventricular dysplasia (ARVD) patients. Methods: MRI of 38 first-degree relatives was analyzed for abnormalities blinded to the mutation status. Focal crinkling of the right ventricle (accordion sign) was observed in 15/25 mutation positive vs. 0/13 mutation negative patients. The presence of this finding was sought in 207 patients evaluated for ARVD blinded to clinical history and was correlated with electrophysiologic findings. Results: Mean age of the population was 32±16, 61% had a family history of ARVD. Based on non-invasive testing the 207 patients were classified into 4 groups 1) 106 normal, 2) 57 Idiopathic ventricular tachycardia (VT), 3) 33 probable ARVD, 4) 11 definite ARVD. A family history of ARVD was present in 74%, 0%, 67% and 36% respectively. 43 patients had the “accordion sign” and the prevalence in the four groups was 10%, 5%, 70% and 100% respectively. The abnormality was seen in the sub-tricuspid region in 62%, outflow tract in 30% in both regions in 8%. After excluding group 4, 48% of patients with the accordion sign had T wave inversion’s beyond V1 compared with 7% of those without the sign (p<0.001). During electrophysiologic testing, reentrant VT could be induced in 44% (7/16) of patients with accordion sign compared to 2% (1/48) without the sign (p<0.01). Conclusion: Focal crinkling of the RV termed “Accordion sign” may represent an early manifestation of ARVD that correlates with mutation positive status and predicts inducibility of ventricular arrhythmias.

Family history of a patient with arrhythmogenic right ventricular dysplasia (ARVD) and coincidence with other malformations

Resuscitation ◽  
1993 ◽  
Vol 25 (1) ◽  
pp. 88
Author(s):  
Robert Zweiker ◽  
Bernd Eber ◽  
Martin Schumacher ◽  
Johann Dusleag ◽  
Brigitte Rotman ◽  
...  
Keyword(s):  
Family History ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Right Ventricular Dysplasia ◽  
History Of

Magnetic Resonance Imaging Reveals Novel Phenotypic Expression of Desmosomal Mutation in First-Degree Relatives of Patients with Arrhythmogenic Right Ventricular Dysplasia (ARVD). Prevalence and Clinical Significance in Patients Evaluated For ARVD

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Harikrishna Tandri
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Clinical Significance ◽  
Phenotypic Expression ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Resonance Imaging ◽  
First Degree Relatives ◽  
Right Ventricular Dysplasia ◽  
Johns Hopkins University

Abstract 1768 Harikrishna Tandri, Darshan Dalal, Aditya Jain, Daniel P Judge, Theodore Abraham, Cynthia James, Samanthapudi K Daya, Crystal Tichnell, Stuart Russell, Hugh Calkins, David A Bluemke, Johns Hopkins University, Baltimore, MD Harikrishna Tandri, 2007 Finalist and Presenting Author

scholarly journals A clinical case of arrhythmogenic right ventricular dysplasia

2021 ◽  
Vol 2 (1) ◽  
pp. 109-114
Author(s):  
E. V. Solovyoval ◽  
N. A. Popova ◽  
T. V. Vlasoval ◽  
M. L. Gorbunova ◽  
L. N. Antsygina
Keyword(s):  
Right Ventricular ◽  
Clinical Case ◽  
Ventricular Myocardium ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Myocardial Diseases ◽  
Conduction Disorders ◽  
Right Ventricular Dysplasia ◽  
Common Causes ◽  
The Right

Arhythmogenic right ventricular dysplasia (ADP) refers to hereditary myocardial diseases, in which there are structural and functional disorders in the right ventricular myocardium, causing rhythm and conduction disorders, including fatal ventricular arrhythmias. ADP is considered one of the most common causes of sudden cardiac death in young people and people who are engaged in sports. However, in practice, there are cases of this disease in people of an older age category. Diagnosis of ADP is still difficult due to the possible long-term asymptomatic course of the disease. The article describes a clinical case of ADP in a 48-year-old man.

scholarly journals Isolated Myocardial Infarction of the Right Ventricle Complicated by Myocardial Rupture with Cardiac Tamponade in a Female Patient with Arrhythmogenic Right Ventricular Dysplasia

Kardiologiia ◽  
2019 ◽  
Vol 59 (6) ◽  
pp. 86-90
Author(s):  
M. N. Baranova ◽  
M. Yu. Ogarkov ◽  
A. E. Skripchenko ◽  
F. N. Chavdar ◽  
A. Yu. Yankin ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Cardiac Tamponade ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Transmural Infarction ◽  
Right Ventricular Dysplasia ◽  
Anterolateral Wall ◽  
Myocardial Rupture ◽  
The Right

Arrhythmogenic dysplasia of the right ventricle is a rare pathology of the myocardium, the diagnosis of which is difficult. Isolated myocardial infarction of the right ventricle occurs and is diagnosed extremely rarely. In this article we describe a case of arrhythmogenic right ventricular dysplasia, complicated by transmural infarction of the anterolateral wall of the right ventricle, myocardial rupture, and cardiac tamponade.

scholarly journals Sudden Death in a Middle-Aged Gentleman

2020 ◽  
Vol 5 (1) ◽  
Author(s):  
Selvaraja GR
Keyword(s):  
Right Ventricular ◽  
Cause Of Death ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Late Presentation ◽  
Prior History ◽  
Medical Illness ◽  
Unexpected Death ◽  
Right Ventricular Dysplasia ◽  
Fatty Replacement ◽  
The Right

A sudden unexpected death in a healthy individual often leads to panic within a community and it’s frequently associated with malicious mischief. Suicide, infections, genetic or congenital abnormalities may well lead to sudden deaths. All these possibilities are vital in determining the cause of death and facilitate investigation, hence must be considered judiciously. Here, we report a case of a 49-year-old gentleman without underlying medical illness or prior history of heart disease found dead in his home by his wife. Postmortem and histopathology findings determined the cause of death. External examination revealed no obvious injuries whereas 50% narrowing was disclosed in the left anterior descending artery at internal examination. Histology finding showed fibro-fatty replacement of the right ventricle myocardium. Toxicological analyses were unrewarding. It was concluded that the cause of death was arrhythmogenic right ventricular dysplasia. In conclusion, the findings of the forensic autopsy and histopathology facilitated the investigation and established the cause of death. The cause of death attributed to arrhythmogenic right ventricular dysplasia (ARVD). This case demonstrates ARVD can have a late presentation and it should always be borne in mind as a potential cause of death, necessitating this case report.

Abstract 13804: Correlation between Electrocardiographic Features and Local Activation Pattern in Arrhythmogenic Right Ventricular Dysplasia

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Tanyanan Tanawuttiwat ◽  
Anneline S Te Riele ◽  
Binu Philips ◽  
Cynthia A James ◽  
Brittney Murray ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Anterior Wall ◽  
Activation Pattern ◽  
S Wave ◽  
Basal Angle ◽  
Right Ventricular Dysplasia ◽  
Surface Ecg ◽  
Qrs Duration ◽  
The Right

Background: Depolarization abnormalities in the terminal portion of the QRS are frequently seen in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C). The purpose of this study was to correlate the electroanatomic activation pattern of the RV endocardium and epicardium to the surface ECG. Methods: Thirty consecutive ARVD/C patients (Mean age 33.1 +/- 11.2 years, 16 (53%) men) underwent detailed endocardial and epicardial electroanatomical mapping (EAM). Local sinus rhythm activation was annotated at the sharpest intrinsic deflection of the bipolar electrogram, including late potentials. ECG features were classified into 5 major patterns; 1. Normal QRS (12 patients) 2. Epsilon wave (5 patients) 3. Incomplete RBBB (5 patients) 4. Atypical complete RBBB (6 patients) and 5. Prolonged terminal activation duration (TAD) (2 patients) Results: The earliest endocardial and epicardial RV activation occurred on the mid anteroseptal wall on all ECG patterns. Figure 1 represented activation area (purple) after the QRS or during the delayed depolarization phase. Nearly all endocardial and epicardial RV was activated well within the QRS duration in patients with normal QRS but was activated during R’ in patients with CRBBB. The delayed activation during Epsilon wave consistently occurred in basal anterior wall and basal angle of RV. In patients with TAD, the activation of RVOT and basal angle RV represented slurred S wave. Conclusion: ECG features in ARVD/C are correlated with late activation in specific regions of RV and total endocardial activation time. The delay activation of basal anterior wall and basal angle of RV represents the Epsilon wave in the right precordial ECG.

scholarly journals Arrhythmogenic Right Ventricular Dysplasia in Neuromuscular Disorders

2016 ◽  
Vol 10 ◽  
pp. CMC.S38446 ◽  
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger
Keyword(s):  
Right Ventricular ◽  
Ryanodine Receptor ◽  
Transmembrane Protein ◽  
Genetic Disorder ◽  
Neuromuscular Disorders ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Lamin A ◽  
Right Ventricular Dysplasia ◽  
Dystrophia Myotonica Protein Kinase ◽  
The Right

Objectives Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, genetic disorder predominantly affecting the right ventricle. There is increasing evidence that in some cases, ARVD is due to mutations in genes, which have also been implicated in primary myopathies. This review gives an overview about myopathy-associated ARVD and how these patients can be managed. Methods A literature review was done using appropriate search terms. Results The myopathy, which is most frequently associated with ARVD, is the myofibrillar myopathy due to desmin mutations. Only in a single patient, ARVD was described in myotonic dystrophy type 1. However, there are a number of genes causing either myopathy or ARVD. These genes include lamin A/C, ZASP/cypher, transmembrane protein-43, titin, and the ryanodine receptor-2 gene. Diagnosis and treatment are identical for myopathy-associated ARVD and nonmyopathy-associated ARVD. Conclusions Patients with primary myopathy due to mutations in the desmin, dystrophia myotonica protein kinase, lamin A/C, ZASP/cypher, transmembrane protein-43, titin, or the ryanodine receptor-2 gene should be screened for ARVD. Patients carrying a pathogenic variant in any of these genes should undergo annual cardiological investigations for cardiac function and arrhythmias.

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