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Right ventricle cardiomyopathy

Sanjay Sivalokanathan, Tarek Zghaib, Gabriela V Greenland, Nestor Vasquez, Shibani M Kudchadkar, Effrosyni Kontari, Dai-Yin Lu, Ketty Dolores-Cerna, Rob J van der Geest, Ihab R Kamel, Jeffrey E Olgin, Theodore P Abraham, Saman Nazarian, Stefan L Zimmerman, M Roselle Abraham
OBJECTIVES: This study hypothesized that paroxysmal atrial fibrillation (PAF) reflects the presence of a more severe cardiac hypertrophic cardiomyopathy (HCM) phenotype. BACKGROUND: HCM is characterized by myocyte hypertrophy, fibrosis, and a high prevalence of PAF. It is currently unresolved whether atrial fibrillation (AF) is a marker or a mediator of adverse outcomes in HCM. METHODS: This study retrospectively examined 45 HCM patients who underwent cardiovascular magnetic resonance (CMR) imaging in sinus rhythm...
March 2019: JACC. Clinical Electrophysiology
Silvia Castelletti, Lia Crotti, Federica Dagradi, Valeria Rella, Sabrina Salerno, Gianfranco Parati, Franco Cecchi
Absence of the pericardium is a rare congenital disease in which the fibroserum membrane covering the heart is partially or totally absent. It is characterized by few echocardiography (ECG) and imaging features that can mislead the diagnosis to an inherited cardiac disease, such as arrhythmogenic right ventricular cardiomyopathy. Although it has often a benign course, this congenital defect should be identified as in some cases herniation and strangulation can be life-threatening and cause sudden cardiac death...
March 8, 2019: Clinical Journal of Sport Medicine: Official Journal of the Canadian Academy of Sport Medicine
Philipp Heermann, H Fritsch, M Koopmann, P Sporns, M Paul, W Heindel, E Schulze-Bahr, C Schülke
OBJECTIVES: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS. METHODS: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT...
March 13, 2019: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
Kentaro Yamamoto, Xin Guo, Ken-Ichi Mizutani, Nozomu Kurose, Motona Kumagai, Akihiro Shioya, Satoko Nakada, Toshie Terauchi, Yoshihiko Ikeda, Hirofumi Aoki, Kouji Kajinami, Sohsuke Yamada
We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man's death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate...
2019: Clinical Medicine Insights. Case Reports
Jongkwon Seo, In Hyun Jung, Jin Hye Park, Gwang Sil Kim, Hye Young Lee, Young Sup Byun, Byung Ok Kim, Kun Joo Rhee
AIMS: Several studies have been reported using right ventricular (RV) strain as a method for evaluating RV function in patients with various cardiovascular diseases; however, the clinical relevance of RV strain in dilated cardiomyopathy (DCM) patients with sinus rhythm is unknown. The aim of this study was to investigate the relationship between RV strain and adverse events in DCM patients with sinus rhythm. METHODS AND RESULTS: We enrolled 143 DCM patients with sinus rhythm who had been first diagnosed, evaluated, and followed at Sanggye Paik Hospital between March 2013 and August 2017...
February 22, 2019: European Heart Journal Cardiovascular Imaging
Guo-Liang Li, Guy Fontaine, Jine Wu, Shuanliang Fan, Chaofeng Sun, Ardan M Saguner
Research on atrial histology of humans without cardiovascular disease is scarce. Therefore, our aim was to study human atrial histology in subjects without cardiovascular disease. Histology of the right atrium, left atrium or atrial septum was studied in eight patients (one newborn infant and seven adults) who died of a non-cardiac cause and who were not known to suffer from any cardiovascular pathology. Staining with hematoxylin phloxine saffron or Masson's trichrome was performed to have a better identification of fibrosis and H&E for better identification of lymphocytes...
February 14, 2019: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
Marwan M Refaat, Sylvana Hassanieh, Jad A Ballout, Patrick Zakka, Mostafa Hotait, Athar Khalil, Fadi Bitar, Mariam Arabi, Samir Arnaout, Hadi Skouri, Antoine Abchee, Bernard Abi-Saleh, Maurice Khoury, Andreas Massouras, Georges Nemer
BACKGROUND: Cardiomyopathies affect more than 0.5% of the general population. They are associated with high risk of sudden cardiac death, which can result from either heart failure or electrical abnormalities. Although different mechanisms underlie the various types of cardiomyopathies, a principal pathology is common to all and is usually at the level of the cardiac muscle. With a relatively high incidence rate in most countries, and a subsequent major health burden on both the families and governments, cardiomyopathies are gaining more attention by researchers and pharmaceutical companies as well as health government bodies...
February 14, 2019: BMC Medical Genomics
Holger Burchert, Adam J Lewandowski
PURPOSE OF REVIEW: Around 10% of the global population is born preterm (< 37 weeks' gestation). Preterm birth is associated with an increased risk of cardiovascular events, with preterm-born individuals demonstrating a distinct cardiac phenotype. This review aims to summarize the main phenotypic features of the preterm heart and directions for future research to develop novel intervention strategies. RECENT FINDINGS: Being born between 28 and 31 weeks' gestation results in a 4-fold higher risk of heart failure in childhood and adolescence and 17-fold increased risk when born less than 28 weeks' gestation...
February 14, 2019: Current Treatment Options in Cardiovascular Medicine
Fatemeh Zabihollahy, James A White, Eranga Ukwatta
PURPOSE: Accurate 3-dimensional (3D) segmentation of myocardial replacement fibrosis (i.e. scar) is emerging as a potentially valuable tool for risk stratification and procedural planning in patients with ischemic cardiomyopathy. The main purpose of this study was to develop a semi-automated method using a 3D convolutional neural network (CNN)-based for the segmentation of left ventricle (LV) myocardial scar from 3D late gadolinium enhancement magnetic resonance (LGE-MR) images. METHODS: Our proposed CNN is built upon several convolutional and pooling layers aimed at choosing appropriate features from LGE MR images to distinguish between myocardial scar and healthy tissues of the left ventricle...
February 8, 2019: Medical Physics
Dan Taylor, Walid Habre
Database analysis has proved that peri-operative cardiac arrest occurs with increased frequency in children with congenital heart disease. Several case series and bigger data from ACS NSQIP, have elucidated subgroups at the highest risk. Consistently patients with single ventricle physiology (especially prior to cavo-pulmonary anastomosis), severe/supra-systemic pulmonary hypertension, complex lesions and cardiomyopathy with significantly reduced ventricular function have been shown to be of the greatest concern...
February 1, 2019: Paediatric Anaesthesia
Bianca Olivia Cojan-Minzat, Paul-Mihai Boarescu, Gabriel Cismaru, Radu Rosu, Carmen Cionca, Dana Pop, Dumitru Zdrenghea, Lucia Agoston-Coldea
RTIONALE: Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by the presence of a thin compacted layer of myocardium and a spongy subendocardial layer with trabeculations and recesses. LVNC associated Wolf-Parkinson-White syndrome is very rare. PATIENT CONCERNS: A 32-year-old male presented with short episodes of palpitations and a syncope 6 months before his hospitalization. DIAGNOSIS: His ECG revealed the presence of a right posterior accessory pathway...
February 2019: Medicine (Baltimore)
Erwin K Gudenschwager, Jonathan A Abbott, Tanya LeRoith
Dilated cardiomyopathy (DCM) is a myocardial disease characterized by ventricular chamber dilation associated with systolic myocardial dysfunction in the absence of other cardiac lesions. DCM occasionally develops in conjunction with proliferation of fibroelastic fibers in the endocardium, producing endocardial fibroelastosis (EFE). Although early reports describe EFE as a primary disease, evidence now suggests that EFE may develop as a response to myocardial dysfunction. Echocardiographic evaluation of a 4-wk-old Pallas cat ( Otocolobus manul) with respiratory distress revealed enlargement of both atria, enlarged end-systolic left ventricular dimension, and left ventricular dilation...
January 29, 2019: Journal of Veterinary Diagnostic Investigation
Adolfo Fontenla, María López-Gil, Daniel Rodríguez Muñoz, David Díez de Las Heras, Fernando Arribas
A 45 year-old man with non-ischaemic cardiomyopathy and a single-chamber ICD (with a lead implanted in the right ventricular apex, RVA) for primary prevention was referred for urgent ablation due to multiple shocks (up to 40) secondary to sustained and incessant regular tachycardias despite amiodarone infusion and deep sedation. A previous cardiac magnetic resonance revealed a severely dilated left ventricle, with a left ventricular ejection fraction of 15% and absence of gadolinium late enhancement.
January 28, 2019: Journal of Cardiovascular Electrophysiology
Yi-Feng Li, Adelino V M Canário, Deborah M Power, Marco A Campinho
BACKGROUND: Endocrine disruption is one of the consequences of industrialization and chemicals released into the environment have a profound impact on organisms. Waterborne micromolar concentrations of ioxynil (IOX) and diethylstilbestrol (DES) in fish affect the development of the heart, vasculature and thyroid gland. OBJECTIVES: The present study aimed to determine how IOX and DES disrupt the crosstalk between the developing thyroid gland and cardio-vascular system in zebrafish...
January 24, 2019: Environment International
A Ben Halima, D Kobaa, M Ben Halima, S Ayachi, M Belkhiria, H Addala
INTRODUCTION: Premature ventricular complexes (PVC) are generally considered as a benign electrocardiographic abnormality in the athletic population. However it may be indicative of underlying heart disease which may increase the risk of sudden death. This implies the need for cardiological 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 decision regarding practicing sports...
January 22, 2019: Annales de Cardiologie et D'angéiologie
Adaya Weissler-Snir, Waseem Hindieh, Danna A Spears, Arnon Adler, Harry Rakowski, Raymond H Chan
OBJECTIVES: To examine the relationship between late gadolinium enhancement (LGE) extent and Non-sustained ventricular tachycardia (NSVT) characteristics in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: NSVT has been shown to be independently associated with sudden death (SD) in HCM. Previous studies have found LGE on cardiac magnetic resonance (CMR) to be independently associated with NSVT. METHODS: Seventy-three patients who had 14-day Holter monitoring for either risk stratification for SD (n=62) or evaluation of atrial fibrillation (n=11) a CMR study were included...
January 24, 2019: Journal of Cardiovascular Electrophysiology
Pascal Amedro, Marie Vincenti, Gregoire De La Villeon, Kathleen Lavastre, Catherine Barrea, Sophie Guillaumont, Charlene Bredy, Lucie Gamon, Albano C Meli, Olivier Cazorla, Jeremy Fauconnier, Pierre Meyer, François Rivier, Jerome Adda, Thibault Mura, Alain Lacampagne
BACKGROUND: Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Speckle-tracking echocardiographic (STE) imaging is emerging as a noninvasive functional biomarker to consider in the early detection of DMD-related cardiomyopathy. However, STE analysis has not been assessed in a prospectively controlled study, especially in presymptomatic children with DMD, and no study has used STE analysis in all three displacements (longitudinal, radial, and circumferential) and for both ventricles...
January 21, 2019: Journal of the American Society of Echocardiography
Sartaj S Gill, Mark Doyle, Diane V Thompson, Ronald Williams, June Yamrozik, Saundra B Grant, Robert W W Biederman
BACKGROUND: While left ventricular ejection fraction (LVEF) has been shown to have prognostic value in ischemic cardiomyopathy (ICMX) patients, right ventricular ejection fraction (RVEF) has not been systematically evaluated in either ICMX or non-ischemic cardiomyopathy (NICMX) patients. Moreover, an accurate estimation of RVEF is problematic due to the geometry of the right ventricle (RV). Over the years, there have been improvements in the resolution, image acquisition and post-processing software for cardiac magnetic resonance imaging (CMR), such that CMR has become the "gold standard" for measuring RV volumetrics and RVEF...
January 23, 2019: Diagnostics
Meghan C Hughes, Sofhia V Ramos, Patrick C Turnbull, Brittany A Edgett, Jason S Huber, Nazari Polidovitch, Uwe Schlattner, Peter H Backx, Jeremy A Simpson, Christopher G R Perry
KEY POINTS SUMMARY: -98% of patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy, with 40% developing heart failure -While increased propensity for mitochondrial induction of cell death has been observed in left ventricle, it remains unknown whether this is linked to impaired mitochondrial respiratory control and elevated H2 O2 emission prior to the onset of cardiomyopathy -Classic mouse models of DMD demonstrate hyper-regeneration in skeletal muscle which may mask mitochondrial abnormalities...
January 23, 2019: Journal of Physiology
Jorge Elias Neto, Joelci Tonet, Robert Frank, Guy Fontaine
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) was initially recognized as a clinical entity by Fontaine and Marcus, who evaluated a group of patients with ventricular tachyarrhythmia from a structurally impaired right ventricle (RV). Since then, there have been significant advances in the understanding of the pathophysiology, manifestation and clinical progression, and prognosis of the pathology. The identification of genetic mutations impairing cardiac desmosomes led to the inclusion of this entity in the classification of cardiomyopathies...
January 2019: Arquivos Brasileiros de Cardiologia
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