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https://www.readbyqxmd.com/read/28812984/modelling-the-effects-of-quinidine-disopyramide-and-e-4031-on-short-qt-syndrome-variant-3-in-the-human-ventricles
#1
Cunjin Luo, Kuanquan Wang, Henggui Zhang
Short QT syndrome (SQTS) is an inherited cardiac channelopathy, but at present little information is available on its pharmacological treatment. SQT3 variant (linked to the inward rectifier potassium current IK1) of SQTS, results from a gain-of-function mutation (Kir2.1 D172N) in the KCNJ2-encoded channels, which is associated with ventricular fibrillation (VF). Using biophysically-detailed human ventricular computer models, this study investigated the potential effects of quinidine, disopyramide, and E-4031 on SQT3...
August 16, 2017: Physiological Measurement
https://www.readbyqxmd.com/read/28807863/prenatal-diagnosis-of-complex-phenotype-in-a-13-week-old-fetus-with-an-interstitial-multigene-deletion-20q13-13-q13-2-by-chromosomal-microarray
#2
Feodora Stipoljev, Danka Miric-Tesanic, Tomislav Hafner, Maja Barbalic, Monika Logara, Ruzica Lasan-Trcic, Ana Vicic, Romana Gjergja-Juraski
We report the first trimester three-dimensional ultrasonographic findings in a 13-week-old fetus with complex phenotype and a de novo 4.7 Mb multigene deletion encompassing chromosome region 20q13.13-q13.2 detected by chromosomal microarray. Fetal sonography detected radial-ray anomalies in the form of bilateral absence of thumbs and the left club hand deformity. The presence of single atrioventricular canal instead of the atrial septal defect typical for Holt-Oram syndrome pointed us to rather suspect the SALL4 related diseases...
August 11, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28798531/a-choroid-plexus-cyst-in-the-fourth-ventricle-of-a-sprague-dawley-rat
#3
Atsuko Murai, Kenji Nakamura, Norifumi Takimoto, Kengo Namiki, Daisuke Hibi, Yukihiro Yanagizawa, Koji Shimouchi
Choroid plexus cysts are rare lesions in the brain and are reported in humans and dogs. Herein, we report a choroid plexus cyst found in a 10-week-old female Sprague-Dawley rat. Histologically, a cyst measuring approximately 600 μm in diameter was found in the fourth ventricle of the brain. The cyst was lined with a single layer of flattened cells and was present in the connective tissue of the choroid plexus. Next to the cyst, a dilated tube was found with a similar morphology to the epithelium of the choroid plexus...
July 2017: Journal of Toxicologic Pathology
https://www.readbyqxmd.com/read/28795517/cardiovascular-adaptation-to-the-fontan-circulation
#4
REVIEW
Gruschen R Veldtman, Alexander R Opotowsky, Samuel G Wittekind, Jack Rychik, Daniel J Penny, Mark Fogel, Bradley S Marino, Marc Gewillig
Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations...
August 10, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28791727/morphology-and-classification-of-right-ventricular-bands-in-the-domestic-dog-canis-familiaris
#5
L A Cope
Ventricular bands, also designated as 'false tendons', are described as single or multiple strands that cross the ventricles and have no connection to valvular cusps. Previous work indicates these strands are present in the ventricles of humans and some animal hearts and not always associated with cardiac pathologies. Despite these previous studies, the published literature is limited in documenting the morphology of these strands and incidence in animals. In this study, examination of 89 hearts showed six types of ventricular bands in the right ventricle of the domestic dog...
August 9, 2017: Anatomia, Histologia, Embryologia
https://www.readbyqxmd.com/read/28783102/the-pulmonary-circulation-in-the-single-ventricle-patient
#6
REVIEW
Amanda Hauck, Nicolas Porta, Steven Lestrud, Stuart Berger
In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in 'single ventricle' physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan...
August 7, 2017: Children
https://www.readbyqxmd.com/read/28782496/morphological-three-dimensional-analysis-of-papillary-muscles-in-borderline-left-ventricles
#7
Mari N Velasco Forte, Mohamed Nassar, Nick Byrne, Miguel Silva Vieira, Israel V Pérez, Bram Ruijsink, John Simpson, Tarique Hussain
OBJECTIVE: Mitral valve anatomy has a significant impact on potential surgical options for patients with hypoplastic or borderline left ventricle. Papillary muscle morphology is a major component regarding this aspect. The purpose of this study was to use cardiac magnetic resonance to describe the differences in papillary muscle anatomy between normal, borderline, and hypoplastic left ventricles. METHODS: We carried out a retrospective, observational cardiac magnetic resonance study of children (median age 5...
September 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28775102/life-threatening-airway-bleeding-after-palliation-of-single-ventricle-congenital-heart-disease
#8
Konstantin Averin, Jonathan W Byrnes, Dan T Benscoter, Wendy Whiteside, Holly DeSena, Russel Hirsch, Bryan H Goldstein
OBJECTIVE: To describe acute and mid-term outcomes following presentation with, and treatment for, life-threatening airway bleeding (hemoptysis) in palliated single ventricle congenital heart disease (SV-CHD). METHODS: Case series of patients with SV-CHD who presented to a large congenital heart centre with hemoptysis between 2004 and 2015. RESULTS: Twenty-one episodes of hemoptysis occurred in 12 patients (58% female, median 10.5 (IQR 7.2, 16...
August 3, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28775062/de-novo-fgf12-fibroblast-growth-factor-12-functional-variation-is-potentially-associated-with-idiopathic-ventricular-tachycardia
#9
Qianqian Li, Yuanyuan Zhao, Gang Wu, Shanshan Chen, Yingchao Zhou, Sisi Li, Mengchen Zhou, Qian Fan, Jielin Pu, Kui Hong, Xiang Cheng, Qing Kenneth Wang, Xin Tu
BACKGROUND: Idiopathic ventricular tachycardia (VT) is a type of cardiac arrhythmia occurring in structurally normal hearts. The heritability of idiopathic VT remains to be clarified, and numerous genetic factors responsible for development of idiopathic VT are as yet unclear. Variations in FGF12 (fibroblast growth factor 12), which is expressed in the human ventricle and modulates the cardiac Na(+) channel NaV1.5, may play an important role in the genetic pathogenesis of VT. METHODS AND RESULTS: We tested the hypothesis that genetic variations in FGF12 are associated with VT in 2 independent Chinese cohorts and resequenced all the exons and exon-intron boundaries and the 5' and 3' untranslated regions of FGF12 in 320 unrelated participants with idiopathic VT...
August 3, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28774643/transesophageal-echocardiography-3-dimensional-and-speckle-tracking-together-as-sensitive-markers-for-early-outcome-in-patients-with-left-ventricular-dysfunction-undergoing-cardiac-surgery
#10
Alok Kumar, Goverdhan Dutt Puri, Ajay Bahl
OBJECTIVE: Speckle tracking, when combined with 3-dimensional (3D) left ventricular ejection fraction, might prove to be a more sensitive marker for postoperative ventricular dysfunction. This study investigated early outcomes in a cohort of patients with left ventricular dysfunction undergoing cardiac surgery. DESIGN: Prospective, blinded, observational study. SETTING: University hospital; single institution. PARTICIPANTS: The study comprised 73 adult patients with left ventricular ejection fraction <50% undergoing cardiac surgery using cardiopulmonary bypass...
April 5, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28774381/first-in-human-closed-chest-transcatheter-superior-cavopulmonary-anastomosis
#11
Kanishka Ratnayaka, John W Moore, Rodrigo Rios, Robert J Lederman, Sanjeet R Hegde, Howaida G El-Said
BACKGROUND: In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality. A nonsurgical transcatheter alternative would be attractive...
August 8, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28770308/hemoglobin-level-at-stage-1-discharge-has-no-impact-on-inter-stage-growth-and-stability-in-single-ventricle-infants
#12
Claudia Delgado-Corcoran, Deborah U Frank, Stephanie Bodily, Chong Zhang, Katherine H Wolpert, Kathryn Lucas, Theodore J Pysher, Angela P Presson, Susan L Bratton
Hemoglobin levels (Hgb) of infants with a single ventricle (SV) are traditionally maintained high to maximize oxygen-carrying capacity during stage 1 palliation (S1P), stage 2 palliation (S2P), and between stages (IS). A single-center observational cohort study was performed to determine if red blood cell transfusion during the convalescent phase of the S1P (late S1P transfusion) to achieve higher Hgb is associated with benefits during the IS including improved growth and decreased acute medical events. 137 infants <1 year with SV with SIP undergoing care from January 2008 to June 2015 were retrospectively evaluated...
August 3, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28770306/prenatal-echocardiographic-predictors-of-postnatal-management-strategy-in-the-fetus-with-right-ventricle-hypoplasia-and-pulmonary-atresia-or-stenosis
#13
Li Cao, Zhiyun Tian, Jack Rychik
Fetuses with pulmonary atresia or pulmonary stenosis with intact ventricular septum manifest variable degrees of right ventricle hypoplasia and inadequacy. We studied the relationship between prenatal echocardiographic parameters and their progression through gestation as potential predictors of postnatal single-ventricle or two-ventricle care strategy. Serial fetal echocardiograms of pulmonary atresia (n = 28) or severe pulmonary stenosis (n = 8) and intact ventricular septum were reviewed. Measurements included tricuspid valve and mitral valve diameter and Z scores, degree of tricuspid regurgitation, presence of subaortic stenosis, presence of coronary artery fistulae, and Doppler pulsatility indices in middle cerebral and umbilical artery...
August 2, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28766512/radiofrequency-ablation-of-accessory-pathways-in-a-toddler-with-ebstein-s-anomaly-and-functional-single-ventricle-physiology
#14
Hasan Candaş Kafalı, İsa Özyılmaz, Serkan Ünal, Alper Güzeltaş, Yakup Ergül
No abstract text is available yet for this article.
August 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/28766173/clinical-features-and-long-term-outcomes-of-pediatric-intraventricular-meningiomas-data-from-a-single-neurosurgical-center
#15
Zhicen Li, Hao Li, Yuming Jiao, Ji Ma, Shuo Wang, Yong Cao, Jizong Zhao
The purpose of this study is to investigate the clinical characteristics and long-term outcomes of pediatric patients with intraventricular meningioma. We retrospectively analyzed a total of 30 pediatric patients with intraventricular meningiomas who were surgically treated at our department between January 2005 and June 2016 and analyzed their clinical characteristics and surgical outcomes. Among the 160 pediatric patients with intracranial meningioma, 33 (20.6%) had intraventricular lesions. However, only 30 patients had complete demographic and clinical data...
August 2, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28766006/tissue-level-cardiac-electrophysiology-studied-in-murine-myocardium-using-a-microelectrode-array-autonomic-and-thermal-modulation
#16
Jem D Lane, David Montaigne, Andrew Tinker
Cardiac electrophysiology is regulated by the autonomic nervous system, and this has both pathophysiological, and possibly therapeutic importance. Furthermore, chamber differences in electrophysiology exist between atria and ventricles, yet there have been few direct comparisons. There is substantial literature on ion channel modulation at the single-cell level but less work on how this affects tissue-level parameters. We used a microelectrode array system to explore these issues using murine atrial and ventricular tissue slices...
August 1, 2017: Journal of Membrane Biology
https://www.readbyqxmd.com/read/28765742/clinical-characteristics-and-independent-factors-related-to-long-term-outcomes-in-patients-with-left-isomerism
#17
Sun Hyang Lee, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Hong Gook Lim, Woong Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28765741/experience-with-mechanical-circulatory-support-for-medically-intractable-low-cardiac-output-in-a-pediatric-intensive-care-unit
#18
Jung Bin Park, Jae Gun Kwak, Hong-Gook Lim, Woong-Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28761995/intermediate-term-thrombotic-risk-in-contemporary-total-cavo-pulmonary-connection-for-single-ventricle-circulations
#19
Jenna M Faircloth, Olivia Roe, Tarek Alsaied, Joseph S Palumbo, Alexander Vinks, Gruschen R Veldtman
Despite the common occurrence of thrombosis in Fontan circulations, the mid-term thrombotic risk beyond the first two postoperative years is poorly defined especially in total cavo pulmonary Fontan. This study examines the thrombotic incidence and risk beyond the first 2 years after contemporary Fontan surgery. Using a retrospective cohort study design, 89 Fontan patients, 50 male, were included and evaluated with a median of 8.3 years (IQR 6.8-11.4) follow-up. Hospital records were reviewed for known risk factors of thrombosis, thrombotic events, antiplatelet and anticoagulation management, and basic characteristics...
July 31, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28752570/a-preliminary-comparison-of-two-dimensional-speckle-tracking-echocardiography-and-pressure-volume-loop-analysis-in-patients-with-fontan-physiology-the-role-of-ventricular-morphology
#20
David Steflik, Ryan J Butts, George H Baker, Varsha Bandisode, Andrew Savage, Andrew M Atz, Shahryar M Chowdhury
OBJECTIVE: Speckle tracking echocardiography (STE) may be a useful modality for assessing ventricular performance in patients with single ventricle physiology. However, STE's ability to accurately assess ventricular performance in this population is unknown. The objective of this study was to perform a preliminary comparison of STE measures of myocardial deformation to reference standard measures of function derived from pressure-volume loop (PVL) analysis. DESIGN: This was a secondary analysis of a prospective study investigating PVLs in patients with Fontan physiology...
July 28, 2017: Echocardiography
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