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Right ventricle cardiomyopathy

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...
September 19, 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...
September 5, 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
Jin-Sheng Wang, Yi-Gen Shen, Ri-Peng Yin, Saroj Thapa, Yang-Pei Peng, Kang-Ting Ji, Lian-Ming Liao, Jia-Feng Lin, Yang-Jing Xue
BACKGROUND: Patients with frequent premature ventricular contractions (PVCs) are often symptomatic. Catheter ablation was usually indicated to eliminate symptoms in patients with PVCs-induced cardiomyopathy. Currently, PVCs-ablation is also applied for patients with PVCs and no structural heart diseases (SHD); however, the safety and efficacy of ablation in these patients remains unclear. METHODS: In this retrospective study, data from patients who underwent ablation for PVCs from January 2010 to December 2016 at our hospital was retrieved...
August 31, 2018: BMC Cardiovascular Disorders
Sajya M Singh, Susan A Casey, Allison A Berg, Raed H Abdelhadi, William T Katsiyiannis, Mosi K Bennett, Shannon Mackey-Bojack, Emily R Duncanson, Jay D Sengupta
A novel autosomal-dominant in-frame deletion resulting in a nonsense mutation in the desmoplakin (DSP) gene was identified in association with biventricular arrhythmogenic cardiomyopathy across three generations of a large Caucasian family. Mutations that disrupt the function and structure of desmosomal proteins, including desmoplakin, have been extensively linked to familial arrhythmogenic right ventricular cardiomyopathy (ARVC). Analysis of data from 51 individuals demonstrated the previously undescribed variant p...
July 2018: American Journal of Medical Genetics. Part A
Tetsuya Saito, Shigeru Miyagawa, Koichi Toda, Yasushi Yoshikawa, Satsuki Fukushima, Shunsuke Saito, Daisuke Yoshioka, Yasushi Sakata, Takashi Daimon, Yoshiki Sawa
BACKGROUND: Left ventricle (LV) unloading caused by left ventricular assist device (LVAD) has been shown to enhance reverse LV remodeling in end-stage cardiomyopathy. Several reports consistently suggest that pulsatile-flow LVAD has more profound effects compared to continuous-flow LVAD, though the responsible mechanisms are not fully understood. We hypothesized that arterial pulsatility, being affected by the type of LVAD, may affect microvasculature and functional/pathological LV remodeling in end-stage cardiomyopathy...
August 21, 2018: Artificial Organs
Mounes Aliyari Ghasabeh, Anneline S J M Te Riele, Cynthia A James, H S Vincent Chen, Crystal Tichnell, Brittney Murray, John Eng, Brian G Kral, Harikrishna Tandri, Hugh Calkins, Ihab R Kamel, Stefan L Zimmerman
Purpose To compare epicardial fat in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with that in healthy subjects. Materials and Methods In this retrospective study, cardiac CT scans in 44 patients with ARVD/C (mean age, 39 years ± 12; 23 men) were compared with those in 45 control group participants between January 2008 and July 2015. Volumes of intrathoracic adipose tissue, mediastinal adipose tissue (MAT), and total epicardial adipose tissue (EAT) were quantified. EAT was subdivided into three regions-right ventricular (RV) EAT, left ventricular (LV) EAT, and peri-atrial EAT (atrial EAT)-and normalized to MAT for all regions...
August 21, 2018: Radiology
Yubi Lin, Jiana Huang, Siqi He, Ruiling Feng, ZhiAn Zhong, Yang Liu, Weitao Ye, Xin Li, Hongtao Liao, Hongwen Fei, Fang Rao, Zhixin Shan, Chunyu Deng, Xianzhang Zhan, Yumei Xue, Hui Liu, Bin Zhang, Kejian Wang, Qianhuan Zhang, Shulin Wu, Xiufang Lin
BACKGROUND: Sudden cardiac death (SCD) induced by malignant ventricular tachycardia (MVT) among young adults with right ventricular cardiomyopathy/dysplasia (ARVC/D) is a devastating event. Parts of ARVC/D patients have a mutation in genes encoding components of cardiac desmosomes, such as desmoglein-2 (DSG2), plakophilin-2 and desmoplakin. CASE PRESENTATION: Here we report a potentially pathogenic mutation in the DSG2 gene, which was identified in a family with ARVC/D using Whole Exome Sequencing (WES) and Sanger Sequencing...
August 21, 2018: BMC Medical Genetics
Agata Popielarz-Grygalewicz, Jakub S Gąsior, Aleksandra Konwicka, Paweł Grygalewicz, Maria Stelmachowska-Banaś, Wojciech Zgliczyński, Marek Dąbrowski
To determine whether the echocardiographic presentation allows for diagnosis of acromegalic cardiomyopathy. 140 patients with acromegaly underwent echocardiography as part of routine diagnostics. The results were compared with the control group comprising of 52 age- and sex-matched healthy volunteers. Patients with acromegaly presented with higher BMI, prevalence of arterial hypertension, and glucose metabolism disorders (i.e., diabetes and/or prediabetes). In patients with acromegaly, the following findings were detected: increased left atrial volume index, increased interventricular septum thickness, increased posterior wall thickness, and increased left ventricular mass index, accompanied by reduced diastolic function measured by the following parameters: E'med...
2018: International Journal of Endocrinology
Pugazhendhi Vijayaraman, Mina K Chung, Gopi Dandamudi, Gaurav A Upadhyay, Kousik Krishnan, George Crossley, Kristen Bova Campbell, Byron K Lee, Marwan M Refaat, Sanjeev Saksena, John D Fisher, Dhananjaya Lakkireddy
Traditional right ventricular (RV) pacing for the management of bradyarrhythmias has been pursued successfully for decades, although there remains debate regarding optimal pacing site with respect to both hemodynamic and clinical outcomes. The deleterious effects of long-term RV apical pacing have been well recognized. This has generated interest in approaches providing more physiological stimulation, namely, His bundle pacing (HBP). This paper reviews the anatomy of the His bundle, early clinical observations, and current approaches to permanent HBP...
August 21, 2018: Journal of the American College of Cardiology
Irene A Burger, Christine Lohmann, Michael Messerli, Susan Bengs, Anton Becker, Monika Maredziak, Valerie Treyer, Ahmed Haider, Moritz Schwyzer, Dominik C Benz, Ken Kudura, Michael Fiechter, Andreas A Giannopoulos, Tobias A Fuchs, Christoph Gräni, Aju P Pazhenkottil, Oliver Gaemperli, Ronny R Buechel, Philipp A Kaufmann, Catherine Gebhard
BACKGROUND: Sexual dimorphism in cardiac sympathetic outflow has recently gained attention in the context of Takotsubo cardiomyopathy. Previous studies suggest that there are sex- and age-dependent differences in peripheral autonomic control, however, data on cardiac-specific sympathetic activation in aged women and men are lacking. METHODS AND RESULTS: Regional quantitative analysis of cardiac fluorine-18 (18F)- Dihydroxyphenylalanine (DOPA) uptake was retrospectively performed in 133 patients (69 females, mean age 52...
2018: PloS One
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
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