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Right ventricle cardiomyopathy

Flavio D'Ascenzi, Francesca Anselmi, Pietro Piu, Caterina Fiorentini, Salvatore Francesco Carbone, Luca Volterrani, Marta Focardi, Marco Bonifazi, Sergio Mondillo
OBJECTIVES: The aim of this meta-analysis was to derive normal reference values of biventricular size and function estimated by cardiac magnetic resonance (CMR) in competitive athletes. BACKGROUND: Exercise-induced enlargement of cardiac chambers is commonly observed in competitive athletes. However, ventricular dilatation is also a common phenotypic expression of life-threatening cardiomyopathies. The use of CMR for the exclusion of pathology is growing. However, normal reference values have not been established for athletes...
December 6, 2018: JACC. Cardiovascular Imaging
Kalliopi Keramida, George Lazaros, Petros Nihoyannopoulos
Although hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy worldwide, the criteria for its definition and most of the literature concern the left ventricle, confirming the theory that the right ventricle is the neglected one. Right ventricular (RV) involvement includes structural and functional changes with significant impact on clinical presentation and prognosis. The pattern of RV hypertrophy can be variable with possible dynamic obstruction. Histological findings suggest similar pathogenetic changes in both ventricles supporting the common myopathic process with sarcomeric mutations...
November 30, 2018: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
Utkarsh Kohli, Karen L Pumphrey, Aziez Ahmed, Srikant Das
A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). She was treated with milrinone and epinephrine infusions, mechanical ventilation and listed for heart transplant. She underwent Berlin Heart EXCOR biventricular assist device (BiVAD) placement (30 ml LVAD and 25 ml RVAD pumps). No supraventricular tachycardia (SVT) was inducible or noted during her hospitalization...
November 2018: Journal of Electrocardiology
Camille Girard-Bock, Carla C de Araújo, Mariane Bertagnolli, Thuy-An Mai-Vo, Arul Vadivel, Rajesh S Alphonse, Shumei Zhong, Anik Cloutier, Megan R Sutherland, Bernard Thébaud, Anne Monique Nuyt
Very preterm birth is associated with increased cardiovascular diseases and changes in myocardial structure. The current study aimed to investigate the impact of endothelial colony-forming cell (ECFC) treatment on heart morphological changes in the experimental model of neonatal high oxygen (O2 )-induced cardiomyopathy, mimicking prematurity-related conditions. Sprague-Dawley rat pups exposed to 95% O2 or room air (RA) from day 4 (P4) to day 14 (P14) were randomized to receive (jugular vein) exogenous human cord blood ECFC or vehicle at P14 (n = 5 RA-vehicle, n = 8 RA-ECFC, n = 8 O2 -vehicle and n = 7 O2 -ECFC) and the hearts collected at P28...
November 2018: Physiological Reports
Nivedita Patni, Frank Vuitch, Abhimanyu Garg
Context: Congenital generalized lipodystrophy, type 4 (CGL4) is a rare autosomal recessive disorder caused by CAVIN1 mutations. Patients with CGL4 also have myopathy and cardiomyopathy with a predisposition for sudden death due to ventricular arrhythmias. However, underlying pathology for these morbidities remains unknown. Therefore, we report the first autopsy of a Hispanic boy with CGL4. Case description: Our patient had early onset generalized lipodystrophy, feeding difficulties, myopathy, atlanto-axial dislocation and learning disabilities...
November 21, 2018: Journal of Clinical Endocrinology and Metabolism
Sonia Cortassa, Viviane Caceres, Carlo G Tocchetti, Michel Bernier, Rafael de Cabo, Nazareno Paolocci, Steven J Sollott, Miguel A Aon
KEY POINTS: Hearts from type 2 diabetic animals display perturbations in excitation-contraction coupling, impairing myocyte contractility and delaying relaxation, along with altered substrate consumption patterns. Under high glucose and β-adrenergic stimulation conditions, palmitate can, at least in part, offset left ventricle (LV) dysfunction in hearts from diabetic mice improving contractility and relaxation while restoring coronary perfusion pressure. Fluxome calculations of central catabolism in diabetic hearts show that, in presence of palmitate, there is a metabolic remodeling involving tricarboxylic acid cycle, polyol and pentose phosphate pathways, leading to improved redox balance in cytoplasmic and mitochondrial compartments...
November 21, 2018: Journal of Physiology
Sung Il Im, Hye Bin Gwag, Youngjun Park, Seung-Jung Park, June Soo Kim, Young Keun On, Kyoung-Min Park
BACKGROUND: A high burden of ventricular premature depolarizations (VPDs) has been associated with potentially reversible left ventricular (LV) dysfunction, termed as VPD-induced cardiomyopathy (CMP). However, many patients maintain normal LV function despite a high VPD burden. The purpose of this study was to identify CMP by right ventricle apex (RVa) pacing method in patients with high VPD burden. METHODS: A total of 62 patients (28 male; mean age = 50 ± 15 years) with idiopathic VPDs undergoing ablation were enrolled...
November 20, 2018: Pacing and Clinical Electrophysiology: PACE
Sonia Gioffré, Veronica Ricci, Chiara Vavassori, Clarissa Ruggeri, Mattia Chiesa, Ivana Alfieri, Silvia Zorzan, Marta Buzzetti, Giuseppina Milano, Alessandro Scopece, Laura Castiglioni, Luigi Sironi, Giulio Pompilio, Gualtiero I Colombo, Yuri D'Alessandra
BACKGROUND: Doxorubicin (DOX) is a chemotherapeutic drug limited in its usefulness by an adverse side effect, cardiotoxicity. The mechanisms leading to this detrimental occurrence are not completely clear, and lately many authors focused their attention on the possible role of microRNAs (miRNAs), small regulators of cardiovascular functions, in this phenomenon. Notably, these molecules recently emerged also as potential circulating biomarkers of several cardiac diseases. Thus, the aim of this study was the simultaneous investigation of circulating and cardiac tissue miRNAs expression upon DOX treatment in vivo...
November 16, 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Afef Ben Halima, Manel Ben Halima, Zied BelHadj, Marouene Boukhris, Saida Ayachi, Hayet Ben Salah, Héla Addala, Anis Chaouachi, Donia Kobaa
INTRODUCTION: Premature ventricular complexes (PVC) are generally considered as a benign electrocardiographic abnormality in the athleticpopulation. However it may be indicative of underlying heart disease which may increase the risk of sudden death. This implies the need forcardiological evaluation before indicating the ability to practice competitive sports. AIM: The aim of this study was to evaluate an athlete population with PVC and establish underlying etiologies in order to take a decisionregarding practicing sports...
April 2018: La Tunisie Médicale
Tarek Zghaib, Mimount Bourfiss, Jeroen F van der Heijden, Peter Loh, Richard N Hauer, Harikrishna Tandri, Hugh Calkins, Saman Nazarian, Anneline S J M Te Riele, Stefan L Zimmerman, Birgitta K Velthuis
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that is predominantly known to affect the ventricles. Evidence for atrial involvement remains limited. Therefore, we aimed to characterize atrial involvement in ARVC using functional cardiac magnetic resonance, define the extent of atrial size and function variation attributable to ventricular variables, and identify cardiac magnetic resonance-based predictors of atrial arrhythmias (AA) in ARVC...
September 2018: Circulation. Cardiovascular Imaging
Corinne A Betts, Graham McClorey, Richard Healicon, Suzan M Hammond, Raquel Manzano, Sofia Muses, Vicky Ball, Caroline Godfrey, Thomas M Merritt, Tirsa Westering, Liz O'Donovan, Kim E Wells, Michael J Gait, Dominic J Wells, Damian Tyler, Matthew J Wood
Duchenne muscular dystrophy (DMD) is caused by loss of dystrophin protein, leading to progressive muscle weakness and premature death due to respiratory and/or cardiac complications. Cardiac involvement is characterized by progressive dilated cardiomyopathy, decreased fractional shortening and metabolic dysfunction involving reduced metabolism of fatty acids-the major cardiac metabolic substrate. Several mouse models have been developed to study molecular and pathological consequences of dystrophin deficiency, but do not recapitulate all aspects of human disease pathology and exhibit a mild cardiac phenotype...
October 2, 2018: Human Molecular Genetics
Xuebiao Li, Yu Nie, Hong Lian, Shengshou Hu
BACKGROUND: The histologic difference between alcoholic cardiomyopathy (ACM) and idiopathic dilated cardiomyopathy (IDCM) is unclear. The present study aimed to identify the quantitative pathologic features of ACM compared with IDCM. METHODS: Specimens from 6 regions (anterior left ventricle [LV], lateral LV, inferior LV, interventricular septum [IVS], anterior right ventricle [RV], and inferior RV) were sampled from each explanted heart. Specimens from 4 healthy donor hearts were obtained as normal control...
September 2018: Medicine (Baltimore)
Ronald L Melnick
The National Toxicology Program (NTP) conducted two-year studies of cell phone radiation in rats and mice exposed to CDMA- or GSM-modulated radiofrequency radiation (RFR) at exposure intensities in the brain of rats that were similar to or only slightly higher than potential, localized human exposures from cell phones held next to the head. This study was designed to test the (null) hypothesis that cell phone radiation at non-thermal exposure intensities could not cause adverse health effects, and to provide dose-response data for any detected toxic or carcinogenic effects...
September 19, 2018: Environmental Research
Matthew Cauldwell, Philip Steer, Monique Sterrenburg, Suzanne Wallace, Gemma Malin, Gemma Ulivi, Thomas Everett, Adam Daniel Jakes, Catherine E G Head, Aarthi R Mohan, Sophie Haynes, Margaret Simpson, Janet Brennand, Mark R Johnson
OBJECTIVE: To assess median and percentile birthweight distribution in women with various groups of heart disease relative to a contemporaneous comparison group. METHODS: Data on birth weight and gestational age were collected from 1321 pregnancies ≥24 weeks' gestation in 1053 women with heart disease from seven UK maternity units. Women were assigned to one of 16 groups according to their cardiac lesion. In units where it was possible, data on two births, one delivering before and one after index cases, were collected, giving 2307 comparators...
September 21, 2018: Heart: Official Journal of the British Cardiac Society
Patrick Klein, Pierfranceso Agostoni, Wim-Jan van Boven, Rob J de Winter, Martin J Swaans
OBJECTIVES: Adverse remodelling of the left ventricle (LV) after myocardial infarction (MI) results in a pathological increase in LV volume and reduction in LV ejection fraction (EF). We describe the preliminary results of a novel, multicentre, combined transcatheter and minimally invasive technique to reconstruct the remodelled LV by plication and exclusion of the scar, and to reduce the excess volume, resulting in decreased wall stress and increased EF. METHODS: A novel hybrid transcatheter technique that relies on microanchoring technology (Revivent TC™ System, BioVentrix Inc...
September 17, 2018: Interactive Cardiovascular and Thoracic Surgery
Tuncay Kırış, Eyüp Avcı
OBJECTIVE: To investigate whether repetitive measurements of speckle tracking echocardiography (STE)-derived strain (S) and strain rate (SR) could reveal changes in left ventricular function in patients with nonischemic dilated cardiomyopathy treated with levosimendan. METHODS: We included 22 consecutive patients (age 53 ±12 years) with an ejection fraction (EF) below 35% and New York Heart Association (NYHA) class III-IV symptoms that required intravenous inotropic support despite optimal medical therapy...
October 2018: Journal of Clinical Ultrasound: JCU
L M Kozhevnikova, I B Tsorin, V N Stolyaruk, I F Sukhanova, M B Vititnova, T D Nikiforova, L G Kolik, S A Kryzhanovskii
The expression of Epac proteins (exchange protein directly activated by cAMP) and calmodulin (CaM) was assessed by the content of the corresponding mRNA in biopsy specimens of cardiac atrium, left ventricle, and thoracic aorta of rats with alcoholic cardiomyopathy. In the myocardium, overexpression of Еpac1, Ерас2, and СаМ mRNA was found. The content of Epac2 mRNA in the left ventricle was elevated by 2.9 times (p=0.000001), in the left atrium by 3.2 times (p=0.00001), in the right atrium by 3 times (p=0...
September 2018: Bulletin of Experimental Biology and Medicine
Giovanni Donato Aquaro, Alessandro Pingitore, Gianluca Di Bella, Paolo Piaggi, Raffaella Gaeta, Crysanthos Grigoratos, Alessandro Altinier, Adriana Pantano, Elisabetta Strata, Raffaele De Caterina, Gianfranco Sinagra, Michele Emdin
We sought to evaluate the prognostic role of cardiac magnetic resonance (CMR) in patients with definite, borderline and possible diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) as defined by the International Task Force (TF) in 2010. CMR was performed in 175 patients: 52 with definite, 50 with borderline and 73 possible ARVC. Abnormal-CMR was defined as the presence of ≥1 CMR abnormalities (including abnormalities of right ventricular and left ventricular wall motion, fat infiltration, late gadolinium enhancement, dilation and dysfunction of either ventricles)...
August 21, 2018: American Journal of Cardiology
Gang Li, Aditi Khandekar, Tiankai Yin, Stephanie C Hicks, Qiusha Guo, Kentaro Takahashi, Catherine E Lipovsky, Brittany D Brumback, Praveen K Rao, Carla J Weinheimer, Stacey L Rentschler
Several inherited arrhythmias, including Brugada syndrome and arrhythmogenic cardiomyopathy, primarily affect the right ventricle and can lead to sudden cardiac death. Among many differences, right and left ventricular cardiomyocytes derive from distinct progenitors, prompting us to investigate how embryonic programming may contribute to chamber-specific conduction and arrhythmia susceptibility. Here, we show that developmental perturbation of Wnt signaling leads to chamber-specific transcriptional regulation of genes important in cardiac conduction that persists into adulthood...
October 2018: Journal of Molecular and Cellular Cardiology
Sandra Arvidsson, Michael Y Henein, Gerhard Wikström, Ole B Suhr, Per Lindqvist
BACKGROUND: The extent of right ventricular (RV) involvement in transthyretin amyloidosis (ATTR) is unknown. OBJECTIVES: This study sought to establish the degree of RV involvement in ATTR amyloidosis, and compare findings with RV involvement in hypertrophic cardiomyopathy (HCM). METHODS: Forty-two patients with ATTR amyloidosis and echocardiographic evidence of cardiac amyloidosis (cardiac ATTR), 19 ATTR patients with normal left ventricular (LV) wall thickness (non-cardiac ATTR), 25 patients with diagnosed HCM and 30 healthy controls were included in this study...
September 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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