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arrhythmogenic dysplasia

Yu I Pigolkin, M A Shilova, E M Kil'dyushov, Eksp Gal'chikov
The objective of the present study was to analyze the causes of sudden death in the general population and in the fraction of the young subjects (below 39 years of age). The results of the original retrospective study were obtained in the course of forensic medical autopsies of the subjects aged below 39 years that had been carried out during a period of 10 years. It was shown that one of the causes behind sudden death among the young subjects is cardiovascular disorders associated with the pre-existing pathological condition in the form of connective tissue dysplasia...
2016: Sudebno-meditsinskaia Ekspertiza
Elnur Alizade, Mahmut Yesin, Mehmet Vefik Yazicioğlu, Ekrem Bilal Karaayvaz, Adem Atici, Şükrü Arslan, Anıl Avci, Göksel Acar, Mustafa Tabakci, Servet Izci, Selçuk Pala
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by progressive replacement of ventricular myocytes with variable amounts of fibrous and adipose tissue. Several studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (Tp-e) may correspond to the transmural dispersion of repolarization and that increased Tp-e interval and Tp-e/QT ratio are associated with malignant ventricular arrhythmias. The aim of this study was to evaluate repolarization dispersion measured from the 12-lead surface electrocardiogram (including Tp-e interval, Tp-e/QT, and Tp-e/QTc ratio) in asymptomatic ARVD patients METHODS: We selected 27 patients with asymptomatic ARVD and 27 age- and gender-match young, healthy volunteers...
March 28, 2016: Annals of Noninvasive Electrocardiology
Elnur Alizade, Mahmut Yesin, Mehmet Mustafa Tabakci, Anıl Avci, Mustafa Bulut, Göksel Acar, Zeki Şimşek, Servet Izci, Süleyman Barutçu, Selçuk Pala
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by the progressive replacement of ventricular myocytes with variable amounts of fibrous and adipose tissue. Several studies have suggested that speckle tracking echocardiographic (STE) parameters such as strain (S) and strain rate (SR) may prove useful in the early detection of right ventricular (RV) dysfunction. Therefore, the aim of this study was to evaluate RV myocardial function using the STE method in both asymptomatic and symptomatic patients with ARVD and to assess its potential role in the differential diagnosis of these two presentations...
September 7, 2016: Echocardiography
M J van der Pols, T P Mast, P Loh, J F van der Heijden, M J Cramer, R N Hauer, A S J M Te Riele
PURPOSE: With the increased use of genetic testing for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), this disease is being increasingly recognised among elderly patients. However, elderly ARVD/C patients were underrepresented in prior cohorts. We aimed to describe the phenotypical characteristics and outcomes among ARVD/C patients surviving ≥50 years. METHODS: We assessed detailed phenotypical data of 29 patients who (1) presented at ≥50 years of age; and (2) fulfilled 2010 Task Force Criteria (TFC) for ARVD/C by last follow-up...
August 31, 2016: Netherlands Heart Journal
Mimount Bourfiss, Anneline S J M Te Riele, Thomas P Mast, Maarten J Cramer, Jeroen F VAN DER Heijden, Toon A B VAN Veen, Peter Loh, Dennis Dooijes, Richard N W Hauer, Birgitta K Velthuis
INTRODUCTION: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is associated with desmosomal mutations. Although desmosomal disruption affects both ventricles and atria, little is known about atrial involvement in ARVD/C. OBJECTIVE: To describe the extent and clinical significance of structural atrial involvement and atrial arrhythmias (AA) in ARVD/C stratified by genotype. METHODS: We included 71 patients who met ARVD/C Task Force Criteria and underwent cardiac magnetic resonance (CMR) imaging and molecular genetic analysis...
October 6, 2016: Journal of Cardiovascular Electrophysiology
Cira R T di Gioia, Carla Giordano, Bruna Cerbelli, Annalinda Pisano, Elena Perli, Enrico De Dominicis, Barbara Poscolieri, Vincenzo Palmieri, Costantino Ciallella, Paolo Zeppilli, Giulia d'Amati
Nonischemic Left Ventricular Scar (NLVS) is a pattern of myocardial injury characterized by midventricular and/or subepicardial gadolinium hyper enhancement at cardiac magnetic resonance, in absence of significant coronary artery disease. We aimed to evaluate the prevalence of NLVS in juvenile sudden cardiac death and to ascertain its aetiology at autopsy. We examined 281 consecutive cases of sudden death of subjects aged 1 to 35 years of age. NLVS was defined as a thin, grey rim of subepicardial and/or midmyocardial scar in the left ventricular free wall and/or the septum, in absence of significant stenosis of coronary arteries...
August 25, 2016: Human Pathology
Frank L Marcus
No abstract text is available yet for this article.
November 2016: Trends in Cardiovascular Medicine
Jinzhu Hu, Shan Zeng, Qiongqiong Zhou, Wengen Zhu, Zhenyan Xu, Jianhua Yu, Kui Hong
BACKGROUND: At present, the role of ventricular tachycardia (VT) non-inducibility after ablation in patients with non-ischemic cardiomyopathy (NICM) remains controversial. We conducted a meta-analysis of the published literature to assess whether VT non-inducibility after ablation could predict reduced VT recurrence and mortality in patients with NICM. METHODS: PubMed, ScienceDirect, and the Cochrane library were searched for studies evaluating the effects of VT non-inducibility after catheter ablation on the long-term outcome in NICM patients with sustained VT...
November 1, 2016: International Journal of Cardiology
Vanesa Bruña, Pablo Díez-Villanueva, Manuel Martínez-Sellés, Tomás Datino, Francisco Fernández-Avilés
No abstract text is available yet for this article.
August 8, 2016: Revista Española de Cardiología
Asaad A Khan, Lucy Safi, Malissa Wood
Athletic heart syndrome refers to the physiological and morphological changes that occur in a human heart after repetitive strenuous physical exercise. Examples of exercise-induced changes in the heart include increases in heart cavity dimensions, augmentation of cardiac output, and increases in heart muscle mass. These cardiac adaptations vary based on the type of exercise performed and are often referred to as sport-specific cardiac remodeling. The hemodynamic effects of endurance and strength training exercise lead to these adaptations...
April 2016: Methodist DeBakey Cardiovascular Journal
Patrizia Pedrotti, Angelica Peritore, Alberto Cereda, Cristina Giannattasio, Massimo Imazio
We report the incidental finding of pericardial agenesis in a patient with electrocardiographic and echocardiographic abnormalities mimicking arrhythmogenic right ventricular dysplasia. The anatomic findings were clearly depicted by cardiac magnetic resonance. The patient was asymptomatic and the clinical relevance of this finding would be raised in case a possible differential diagnosis of right heart overload should be necessary. Diagnosis may be easily suspected from the ECG and echocardiography; MRI will provide definite diagnosis...
July 27, 2016: Journal of Cardiovascular Medicine
John Garcia, Jackie Tahiliani, Nicole Marie Johnson, Sienna Aguilar, Daniel Beltran, Amy Daly, Emily Decker, Eden Haverfield, Blanca Herrera, Laura Murillo, Keith Nykamp, Scott Topper
Advances in DNA sequencing have made large, diagnostic gene panels affordable and efficient. Broad adoption of such panels has begun to deliver on the promises of personalized medicine, but has also brought new challenges such as the presence of unexpected results, or results of uncertain clinical significance. Genetic analysis of inherited cardiac conditions is particularly challenging due to the extensive genetic heterogeneity underlying cardiac phenotypes, and the overlapping, variable, and incompletely penetrant nature of their clinical presentations...
2016: Frontiers in Cardiovascular Medicine
Golukhova E Z, Gromova O I, Shomahov R A, Bulaeva N I, Bockeria L A
The abrupt cessation of effective cardiac function that is generally due to heart rhythm disorders can cause sudden and unexpected death at any age and is referred to as a syndrome called "sudden cardiac death" (SCD). Annually, about 400,000 cases of SCD occur in the United States alone. Less than 5% of the resuscitation techniques are effective. The prevalence of SCD in a population rises with age according to the prevalence of coronary artery disease, which is the most common cause of sudden cardiac arrest...
April 2016: Acta Naturae
Bálint Lakatos, Attila Kovács, Márton Tokodi, Alexandra Doronina, Béla Merkely
Accurate assessment of right ventricular geometry and function is of high clinical importance. However, several limitations have to be taken into consideration if using conventional echocardiographic parameters. Advanced echocardiographic techniques, such as speckle-tracking analysis or 3D echocardiography are reliable and simple tools providing a cost-effective and non-invasive alternative of current modalities used to characterize the right ventricle. There is a growing interest in the diagnostic and prognostic value of these methods regarding pathological (right ventricular infarction, pulmonary hypertension, arrhythmogenic right ventricular dysplasia, follow-up of heart transplantation) and even physiological (athlete's heart) alterations of the right ventricle...
July 2016: Orvosi Hetilap
Stephan Kemmner, Hasema Lesevic, Tilko Reents, Heribert Schunkert, Christof Burgdorf
A middle-aged female suffering from ARVD presented for routine follow-up 8 weeks after right ventricular radiofrequency ablation of recurring ventricular tachycardia. Echocardiography revealed two right ventricular thrombi in the scar area of right ventricular radiofrequency ablation. Ablation-related thromboembolic events should be considered as possible complication in patients suffering from ARVD.
June 2016: Clinical Case Reports
Richard N Hauer
No abstract text is available yet for this article.
July 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Ákos Bicsák, Alice Jellen, Herwig Tuppy, Wolfgang Paul Poeschl
BACKGROUND: Oral squamous cell carcinoma is the sixth most frequent malignancy in Austria. The incidence of arrhythmogenic right ventricle dysplasia (ARVC), an important cause of sudden cardiac death, is estimated at 1:5000 to 1:1000. CASE REPORT: We present a case of a 75-year-old woman who underwent major oncologic surgery for T4aN0M0 maxillary squamous cell carcinoma and reconstruction with a scapular-latissimus dorsi microvascular flap. The patient died suddenly during her postoperative care...
June 29, 2016: Oral and Maxillofacial Surgery
Jeffrey J Silbiger, Aditya Parikh
Patients with pectus excavatum (PEX) may be referred for echocardiographic examination for a variety of complaints including exercise intolerance, dyspnea, palpitations, or chest pain. It is therefore important for the echocardiographer to have an appreciation of the various abnormalities associated with this disorder. Echocardiographic imaging may reveal a number of structural alterations of the right ventricle as well as a reduction in right ventricular systolic function. Interestingly, a number of these abnormalities have also been described in patients with arrhythmogenic right ventricular dysplasia, although patients with PEX do not share a predilection for malignant ventricular arrhythmias...
August 2016: Echocardiography
Paola Sormani, Alberto Roghi, Alberto Cereda, Angelica Peritore, Angela Milazzo, Giuseppina Quattrocchi, Cristina Giannattasio, Patrizia Pedrotti
INTRODUCTION: Partial anomalous pulmonary venous return (PAPVR) is an uncommon cause of right ventricular dilation. It may be difficult to identify and often remains undiagnosed. METHODS: We reviewed the database of the Cardiac Magnetic Resonance (CMR) Laboratory of Niguarda Hospital, in order to identify the cases of PAPVR between 2008 and 2014. RESULTS: On a total number of 7832 CMR scans, we identified 24 patients with PAPVR (14 male, age 41 ± 18 y) corresponding to 0...
July 2016: Congenital Heart Disease
Elena Vasichkina, Hermine Poghosyan, Lubov Mitrofanova, Roman Tatarsky, Dmitry Lebedev
: Purpose This study aimed to assess the results of endomyocardial biopsy from the right ventricle to establish the possible cause for drug-refractory arrhythmias in children. Materials and methods We enrolled 19 consecutive young patients with drug-refractory arrhythmia, from 2010 to 2013, who underwent endomyocardial biopsy. Inclusion criteria were as follows: age <18 years with a structurally normal heart or mild changes in a structure of the heart initially diagnosed as arrhythmia-induced cardiomyopathy...
May 23, 2016: Cardiology in the Young
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