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Right ventricle cardiomyopathy

Estelle Gandjbakhch, Alban Redheuil, Françoise Pousset, Philippe Charron, Robert Frank
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiomyopathy that can lead to sudden cardiac death and heart failure. Our understanding of its pathophysiology and clinical expressivity is continuously evolving. The diagnosis of ARVC/D remains particularly challenging due to the absence of specific unique diagnostic criteria, its variable expressivity, and incomplete penetrance. Advances in genetics have enlarged the clinical spectrum of the disease, highlighting possible phenotypes that overlap with arrhythmogenic dilated cardiomyopathy and channelopathies...
August 14, 2018: Journal of the American College of Cardiology
Kristen Nelson McMillan, Narutoshi Hibino, Emily E Brown, Rajeev Wadia, Elizabeth A Hunt, Christi Marshall, Mayuri Alvarez-Machado, Diane Alejo, John D Coulson, William Ravekes, Luca A Vricella
OBJECTIVE: While pediatric HeartWare HVAD application has increased, determining candidacy and timing for initiation of pediatric VAD support has remained a challenge. We present our experience with a systematic approach to HVAD implantation as a bridge to pediatric heart transplantation. METHODS: We performed a retrospective, single center review of pediatric patients (n=11) who underwent HVAD implantation between September 2014 and January 2018. Primary endpoints evaluated were survival to heart transplantation, need for right ventricular assist device (RVAD) at any point, ongoing HVAD support or death...
August 7, 2018: Artificial Organs
Rene L Begay, Sharon L Graw, Gianfranco Sinagra, Angeliki Asimaki, Teisha J Rowland, Dobromir B Slavov, Katherine Gowan, Kenneth L Jones, Francesca Brun, Marco Merlo, Daniela Miani, Mary Sweet, Kalpana Devaraj, Eric P Wartchow, Marta Gigli, Ilaria Puggia, Ernesto E Salcedo, Deborah M Garrity, Amrut V Ambardekar, Peter Buttrick, T Brett Reece, Michael R Bristow, Jeffrey E Saffitz, Luisa Mestroni, Matthew R G Taylor
OBJECTIVES: The purpose of this study was to assess the phenotype of Filamin C (FLNC) truncating variants in dilated cardiomyopathy (DCM) and understand the mechanism leading to an arrhythmogenic phenotype. BACKGROUND: Mutations in FLNC are known to lead to skeletal myopathies, which may have an associated cardiac component. Recently, the clinical spectrum of FLNC mutations has been recognized to include a cardiac-restricted presentation in the absence of skeletal muscle involvement...
April 2018: JACC. Clinical Electrophysiology
Joana Ribeiro, Olinda Rebelo, Ana Fernández-Marmiesse, Luís Negrão
A group of heterogeneous muscle diseases are caused by dystrophin gene ( DMD ) mutations. We hereby present a male patient with a diagnosis of symptomatic dilated cardiomyopathy at 44 years-old who developed, soon after, weakness of distal right upper limb. At the age of 58, neurological examination revealed severe atrophy of right thenar muscles, flexion contractures on the right elbow, wrist and fingers, bilateral calf hypertrophy, myotatic areflexia in the upper limbs and hyporeflexia in the lower limbs...
June 2018: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
Magdalena Chottova Dvorakova, Eliska Mistrova, Renate Paddenberg, Wolfgang Kummer, Jana Slavikova
Substance P (SP) is a neuropeptide engaged in the signal transmission of neural C fibers afferents in the myocardium. The actions of SP in the heart are extensive and they are mediated by the neurokinin 1 receptor (NK1R), a member of the tachykinin subfamily of G-protein coupled receptors. The receptors have been found in the heart, but to our knowledge, their exact localization in the heart has not been described yet. Here, we investigated the presence of NK1R protein in separate rat heart compartments by means of western blot and its tissue distribution by means of immunofluorescence...
2018: Frontiers in Physiology
Waldemar Elikowski, Małgorzata Małek-Elikowska, Dariusz Wróblewski, Natalia Fertała, Magdalena Zawodna, Patrycja Lachowska-Kotowska, Krzysztof Skommer
Treatment of intracardiac thrombi (ICTs) with direct-acting oral anticoagulants (DOACs) constitutes a new challenge for this group of medications. Left ventricular thrombi (LVTs) occur mainly in patients with severe left ventricular dysfunction. Advanced heart failure is characterized by a high prevalence of chronic kidney disease (CKD). Apixaban has the best renal profile among all DOACs. The authors describe a group of 7 patients with LVTs treated with apixaban; 6 of them had CKD. LVTs were screened and monitored during therapy using a sector, 3-dimensional and high frequency linear probes; the latter to exclusively assess the left ventricular apex...
June 27, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Jeffery C Powers, Fabio Recchia
Tachypacing-induced heart failure is a well-established large animal model that recapitulates numerous pathophysiological, structural and molecular features of dilated cardiomyopathy and, more in general, of end-stage congestive heart failure. The left or the right ventricle is instrumented with pacing electrodes to impose supernormal heart rates, usually three times higher than baseline values, for a length of time that typically ranges between 3 and 5 weeks. The animal of choice is the dog, although this protocol has been successfully implemented also in pigs, sheep, and rabbits...
2018: Methods in Molecular Biology
Johan van der Merwe, Filip Casselman, Frank Van Praet
The continuous evolution in robotic-, endoscopic- and trans-catheter cardiac interventions resulted in innovative techniques that simultaneously address left ventricular outflow tract obstruction (LVOTO) and concomitant atrioventricular valve (AVV) pathology in the context of hypertrophic obstructive cardiomyopathy (HOCM). We present our brief report of 13 consecutive HOCM patients with concomitant AVV disease, who underwent endoscopic left ventricular septal myomectomy (LVSM) and AVV surgery by Endoscopic Port AccessTM Surgery (EPAS) between March 1st 2010 and October 31st 2015...
2018: Journal of Visualized Surgery
Linda Koutbi, Baptiste Maille, Michael Peyrol, Jérôme Hourdain, Erwan Salaun, Jean-Claude Deharo, Frédéric Franceschi
PURPOSE: Ablation of premature ventricular complexes (PVC) originating from left sided papillary muscles is challenging. We tested a new approach by performing high-density mapping of PVC. METHODS AND RESULTS: We used a 20-pole deflectable spiral catheter during ablation procedures in 4 consecutive patients. Three presented with mitral valve prolapse, one with dilated cardiomyopathy. PVC burden was 24±13%. The procedures lasted 182±55.4 minutes, including 10±3...
June 26, 2018: Pacing and Clinical Electrophysiology: PACE
Aleyda Benitez-Amaro, Valerie Samouillan, Esther Jorge, Jany Dandurand, Laura Nasarre, David de Gonzalo-Calvo, Olga Bornachea, Gerard Amoros-Figueras, Colette Lacabanne, David Vilades, Ruben Leta, Francesc Carreras, Alberto Gallardo, Enrique Lerma, Juan Cinca, Jose M Guerra, Vicenta Llorente-Cortés
Our aim was to identify biophysical biomarkers of ventricular remodelling in tachycardia-induced dilated cardiomyopathy (DCM). Our study includes healthy controls (N = 7) and DCM pigs (N = 10). Molecular analysis showed global myocardial metabolic abnormalities, some of them related to myocardial hibernation in failing hearts, supporting the translationality of our model to study cardiac remodelling in dilated cardiomyopathy. Histological analysis showed unorganized and agglomerated collagen accumulation in the dilated ventricles and a higher percentage of fibrosis in the right (RV) than in the left (LV) ventricle (P = ...
June 19, 2018: Journal of Cellular and Molecular Medicine
Lingmin Wu, Jingru Bao, Erpeng Liang, Siyang Fan, Lihui Zheng, Zhongpeng Du, Gang Chen, Ligang Ding, Shu Zhang, Yan Yao
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardium disorder that predominantly affects the ventricle. Little is known about atrial involvement. This study aimed to assess atrial involvement, especially the role of genotype on atrium in ARVC. METHODS: The incidence, characterization and predictors of atrial involvement were investigated. Nine known ARVC-causing genes were screened and the correlation between genotype and atrial involvement was assessed...
June 13, 2018: Journal of Cardiovascular Electrophysiology
Duarte Martins, Caroline Ovaert, Diala Khraiche, Nathalie Boddaert, Damien Bonnet, Francesca Raimondi
INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. METHODS: Retrospective case series of all patients <18 years admitted to two CMR units for clinical suspicion of myocarditis from March 2012 to June 2017 who had genetic testing for inherited cardiomyopathies including analysis for known ARVC genes...
May 31, 2018: International Journal of Cardiology
David Prior
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a recognised cause of sudden cardiac death during exercise in young athletes. Competitive exercise is also known to accelerate progression of ARVC and exercise restriction is an important part of disease management. Regular endurance training can induce physiological changes detectable on electrocardiography and imaging which may overlap with pathological findings caused by ARVC, thus making differentiation of athlete's heart from ARVC difficult in some cases...
September 2018: Heart, Lung & Circulation
Won-Jong Park, Jang-Won Son, Chan-Hee Lee, Jung-Hee Lee, Ung Kim, Jong-Seon Park, Dong-Gu Shin
No abstract text is available yet for this article.
May 31, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Moustafa O Elsheshtawy, Ahmed N Mahmoud, Mahmoud Abdelghany, Ida H Suen, Adnan Sadiq, Jacob Shani
BACKGROUND: Hypertrophic cardiomyopathy (HCM) with or without left ventricular apical aneurysm (LVA) had been studied in the past. Midventricular obstruction associated with HCM and LVA is a unique entity that has not been distinguished previously as a separate phenotypic disease in HCM patients. METHODS: A systematic review of Pubmed and Google Scholar was conducted from inception until September 2017 for all observational studies conducted on HCM with midventricular obstruction and LVA...
May 22, 2018: Pacing and Clinical Electrophysiology: PACE
Sophie I Mavrogeni, Flora Bacopoulou, Despoina Apostolaki, George P Chrousos
Sudden cardiac death (SCD) is the nontraumatic death, due to loss of heart function that occurs suddenly and unexpectedly within 6 hours of a previously normal state of health. It is related to intense competitive sports promoting ventricular tachycardia (VT)/ventricular fibrillation (VF) in the presence of underlying abnormal substrate. A serial evaluation of cardiac physiologic changes taking place during training will allow the better understanding of athlete's heart and will facilitate its discrimination from other grey-zone cardiomyopathies...
July 2018: European Journal of Clinical Investigation
Kai Chen, Jiangping Song, Zhen Wang, Man Rao, Liang Chen, Shengshou Hu
BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart disease characterized by fibro-fatty replacement of the myocardium. TTN missense variants were previously reported as a pathogenic factor for ACM. HYPOTHESIS: TTN missense variants are commonly identified in ACM, but have limited effect on the phenotype of ACM. METHODS: We sequenced 15 ACM-related genes in 35 patients who had a heart transplantation and quantified myocardium, and fibrous and adipose tissue in blocks of the explanted heart...
May 2018: Clinical Cardiology
D Y Zhang, C P Zhang, D M Lyu
No abstract text is available yet for this article.
April 24, 2018: Zhonghua Xin Xue Guan Bing za Zhi
Giuseppe Santarpia, Francesco Passafaro, Eugenia Pasceri, Annalisa Mongiardo, Antonio Curcio, Ciro Indolfi
RATIONALE: Inadvertent pacemaker/defibrillator lead placement into the left ventricle is an unusual cardiac device-related complication and its diagnosis is not always easy and often misunderstood. Thromboembolic events are frequently associated with this procedural complication. Percutaneous lead extraction should be performed when diagnosis is made early after device implantation while long-life oral anticoagulation is a wise option when the diagnosis is delayed and the lead is not removed...
May 2018: Medicine (Baltimore)
Takato Mohri, Mike Saji, Tatsuya Murai, Morimasa Takayama
Although atrial fibrillation (AF) often exists in patients with hypertrophic obstructive cardiomyopathy (HOCM), combination of tricuspid regurgitation (TR) and AF after implantation of pacemaker/implantable cardioverter defibrillator (ICD) lead and its optimal management have not been well discussed in this population. Herein, we present a patient with HOCM who subsequently died due to severe heart failure and deterioration of TR following ICD lead placement with AF. Autopsy findings demonstrated that ICD leads were entrapped by anomaly structure in the right atrium and ventricle, which might affect deterioration of TR...
February 2018: Journal of Arrhythmia
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