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Adult congenital heart disease, structural heart disease

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https://www.readbyqxmd.com/read/28062939/early-transverse-tubule-development-begins-in-utero-in-the-sheep-heart
#1
Michelle L Munro, Christian Soeller
The ventricular cardiomyocytes of adult mammals contain invaginations of the plasma membrane known as transverse (t)-tubules. These regular structures are essential for the synchronisation of excitation-contraction (EC) coupling throughout the cell, which is a vital process for cardiac function. T-tubules form a close association with the sarcoplasmic reticulum (SR) to form junctions, where several key proteins involved in EC coupling are localised, including the SR calcium release channels-the ryanodine receptors (RyR)...
January 6, 2017: Journal of Muscle Research and Cell Motility
https://www.readbyqxmd.com/read/27989075/maldi-imaging-mass-spectrometry-as-a-lipidomic-approach-to-heart-valve-research
#2
Peggi M Angel, Ahmed S Bayoumi, Robert B Hinton, Yan Ru Su, David Bichell, John E Mayer, H Scott Baldwin, Richard M Caprioli
BACKGROUND: Valvular disease is characterized in part by lipid deposition, but systematic analysis of the patterns of global lipid expression in healthy and diseased valve tissues are unknown. This is due in part to tissue limitations for lipidomic preparations and technologies for evaluating lipid distribution in tissues. The study aim was to examine the application of matrixassisted laser desorption ionization imaging mass spectrometry (MALDI IMS) to the aortic valve during development and disease, as an approach to detect and map lipids and ultimately better understand valve structure and function...
March 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/27957813/neurocognitive-functioning-in-adults-with-congenital-heart-disease
#3
Dawn Ilardi, Kim E Ono, Rebecca McCartney, Wendy Book, Anthony Y Stringer
OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD...
December 13, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27909479/atrial-fibrillation-ablation-in-adults-with-repaired-congenital-heart-disease
#4
REVIEW
Marta Acena Md, Ignasi Anguera Md PhD, Paolo D Dallaglio Md, Marcos Rodriguez Md, Xavier Sabaté Md PhD
The incidence of atrial fibrillation (AF) in congenital heart disease (CHD) adults has increased in the past decades due to a longer life expectancy of this population where the subjects are exposed to cardiac overflow, overpressure and structural changes for years. The literature regarding AF ablation in repaired CHD adults emphasizes the importance of intracardiac echocardiography (ICE) to perform the transseptal puncture and the ablation procedure in the left atrium (LA), both effectively and safely. In small case control studies, where the predominant congenital cardiomyopathy was the atrial septal defect, the most common strategy for ablation was antral isolation of the pulmonary veins showing results, at one year follow-up, similar to those in the general population...
February 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27860144/the-not-so-innocent-heart-murmur-a-five-year-experience
#5
Shaw-Hua Kueh, Thomas Pasley, Miriam Wheeler, James Pemberton
BACKGROUND: Auckland City Hospital (ACH) established a Heart Murmur Clinic (HMC) with the aim of providing prompt assessment of patients with asymptomatic systolic murmurs. This may lead to early intervention and improved outcomes if significant structural heart disease is detected and reassurance if no significant findings found. Similar clinics for children have proven beneficial; the benefit of a HMC in an adult population has been difficult to determine. OBJECTIVES: To review the clinical demographics and echocardiographic information of patients presenting to our HMC and to assess what proportion had significant structural heart disease and determine the common structural abnormalities in this population...
November 16, 2016: Internal Medicine Journal
https://www.readbyqxmd.com/read/27836946/heart-transplantation-in-adult-congenital-heart-disease
#6
REVIEW
Luke J Burchill
Heart failure (HF) in adult congenital heart disease (ACHD) is vastly different to that observed in acquired heart disease. Unlike acquired HF in which pharmacological strategies are the cornerstone for protecting and improving ventricular function, ACHD-related HF relies heavily upon structural and other interventions to achieve these aims. patients with ACHD constitute a small percentage of the total adult heart transplant population (∼3%), although the number of ACHD heart transplant recipients is growing rapidly with a 40% increase over the last two decades...
December 1, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27751192/cognitive-dysfunction-in-adult-chd-with-different-structural-complexity
#7
Manavi Tyagi, Theodora Fteropoulli, Catherine S Hurt, Shashivadan P Hirani, Lorna Rixon, Anna Davies, Nathalie Picaut, Fiona Kennedy, John Deanfield, Shay Cullen, Stanton P Newman
OBJECTIVE: We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS: A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions...
October 18, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27660579/catheter-ablation-to-treat-supraventricular-arrhythmia-in-children-and-adults-with-congenital-heart-disease-what-we-know-and-where-we-are-going
#8
REVIEW
Patricia E Thomas, Scott L Macicek
BACKGROUND: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. METHODS: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD...
2016: Ochsner Journal
https://www.readbyqxmd.com/read/27650247/neurocognitive-and-executive-functioning-in-adult-survivors-of-congenital-heart-disease
#9
Leda Klouda, Wayne J Franklin, Anita Saraf, Dhaval R Parekh, David D Schwartz
OBJECTIVE: Congenital heart disease (CHD) can affect the developing central nervous system, resulting in neurocognitive and behavioral deficits. Preoperative neurological abnormalities as well as sequelae of the open heart operations required to correct structural abnormalities of the heart contribute to these deficits. There are few studies examining the neurocognitive functioning of adults with CHD. This study sought to investigate multiple domains of neurocognitive functioning in adult survivors of CHD who had childhood cardiac surgery with either moderate or severe disease complexity...
September 21, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27618336/assessment-of-risk-factors-for-a-sustainable-on-table-extubation-program-in-pediatric-congenital-cardiac-surgery-5-year-experience
#10
Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Veronique Dinand, Raja Joshi
OBJECTIVE: To delineate risk factors for failure of extubation in the operating room among pediatric cardiac surgery patients. DESIGN: Prospective, observational study. SETTING: Single center, tertiary care, teaching hospital. PARTICIPANTS: The study comprised 448 congenital cardiac surgery patients who were enrolled for intended extubation in the pediatric cardiac operating room over 5 years. INTERVENTIONS: The airways of enrolled patients were extubated in the operating room if predetermined suitability criteria were met...
December 2016: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27562130/evaluation-of-impedance-cardiography-for-measurement-of-stroke-volume-in-congenital-heart-disease
#11
Mohammed Ebrahim, Sanjeet Hegde, Beth Printz, Mark Abcede, James A Proudfoot, Christopher Davis
Noninvasive measurement of cardiac output (CO) and particularly stroke volume (SV) remain difficult but potentially valuable. These variables can be particularly challenging to measure in children with congenital heart disease (CHD). Impedance cardiography (IC) is a technique shown to be accurate in measuring SV in adults and in children with structurally normal hearts. The ease of use and rapidity of SV measurement using IC makes it potentially attractive for young patients with CHD. Advances in IC technology have led to more sophisticated signal-morphology IC (SMIC) devices that may further improve accuracy...
December 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27528386/impact-of-a-structured-referral-algorithm-on-the-ability-to-monitor-adherence-to-appropriate-use-criteria-for-transthoracic-echocardiography
#12
Steven Promislow, Joseph G Abunassar, Behnam Banihashemi, Benjamin J Chow, Girish Dwivedi, Kasra Maftoon, Ian G Burwash
BACKGROUND: Many free-form-text referral requisitions for transthoracic echocardiography (TTE) provide insufficient information to adequately evaluate their adherence to Appropriate Use Criteria (AUC). We developed a structured referral requisition algorithm based on requisition deficiencies identified retrospectively in a derivation cohort of 1303 TTE referrals and evaluated the performance of the algorithm in a consecutive series of cardiology outpatient referrals. METHODS: The validation cohort comprised 286 consecutive TTE outpatient cardiology referrals over a 2-week period...
August 15, 2016: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/27510170/a-novel-tbx20-loss%C3%A2-of%C3%A2-function-mutation-contributes-to-adult%C3%A2-onset-dilated-cardiomyopathy-or-congenital-atrial-septal-defect
#13
Yi-Meng Zhou, Xiao-Yong Dai, Ri-Tai Huang, Song Xue, Ying-Jia Xu, Xing-Biao Qiu, Yi-Qing Yang
Dilated cardiomyopathy (DCM) is the most prevalent form of primary cardiomyopathy in humans and is a leading cause of heart failure and sudden cardiac death. Genetic abnormalities have been demonstrated to be a major contributor to the development of DCM. However, DCM is a genetically heterogeneous disease, and the genetic basis underlying DCM in a significant proportion of patients remains unclear. In the current study, the coding exons and splicing junction sites of the T‑Box 20 (TBX20) gene, which encodes a T‑box transcription factor essential for cardiac morphogenesis and structural remodeling, were sequenced in 115 unrelated patients with idiopathic DCM, and a novel heterozygous mutation, p...
October 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27380040/comparison-of-automated-brain-volumetry-methods-with-stereology-in-children-aged-2-to-3%C3%A2-years
#14
Kristina N Mayer, Beatrice Latal, Walter Knirsch, Ianina Scheer, Michael von Rhein, Bettina Reich, Jürgen Bauer, Kerstin Gummel, Neil Roberts, Ruth O'Gorman Tuura
INTRODUCTION: The accurate and precise measurement of brain volumes in young children is important for early identification of children with reduced brain volumes and an increased risk for neurodevelopmental impairment. Brain volumes can be measured from cerebral MRI (cMRI), but most neuroimaging tools used for cerebral segmentation and volumetry were developed for use in adults and have not been validated in infants or young children. Here, we investigate the feasibility and accuracy of three automated software methods (i...
September 2016: Neuroradiology
https://www.readbyqxmd.com/read/27367306/mental-disorders-in-adults-with-congenital-heart-disease-unmet-needs-and-impact-on-quality-of-life
#15
Mechthild Westhoff-Bleck, Juliane Briest, Daniela Fraccarollo, Denise Hilfiker-Kleiner, Lotta Winter, Ulrike Maske, Markus A Busch, Stefan Bleich, Johann Bauersachs, Kai G Kahl
OBJECTIVE: In adult congenital heart disease (ACHD), mental health status and quality of life become important issues due to improved life expectancy. Current literature provides conflicting data regarding mental health status in ACHD. Furthermore, none of the studies so far compared prevalence rates with a matched control group. METHODS: The prevalence of mental disorders was assessed in 150 ACHD using a structured interview, and compared to 12-months estimates of the general German population...
November 1, 2016: Journal of Affective Disorders
https://www.readbyqxmd.com/read/27335817/advances-in-the-study-of-heart-development-and-disease-using-zebrafish
#16
Daniel R Brown, Leigh Ann Samsa, Li Qian, Jiandong Liu
Animal models of cardiovascular disease are key players in the translational medicine pipeline used to define the conserved genetic and molecular basis of disease. Congenital heart diseases (CHDs) are the most common type of human birth defect and feature structural abnormalities that arise during cardiac development and maturation. The zebrafish, Danio rerio, is a valuable vertebrate model organism, offering advantages over traditional mammalian models. These advantages include the rapid, stereotyped and external development of transparent embryos produced in large numbers from inexpensively housed adults, vast capacity for genetic manipulation, and amenability to high-throughput screening...
June 2016: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/27280062/right-ventricular-myocardial-ischemia-with-arrhythmia-in-an-asphyxiated-newborn
#17
Anne Lee Solevåg, Georg M Schmölzer, Po-Yin Cheung
Background Infant and neonatal myocardial infarction (MI) has been described in association with congenital heart disease, coronary artery abnormalities, myocarditis, and tumors. MI in the perinatal period in a structurally normal heart and with ventricular arrhythmia as a presenting feature has not been thoroughly described. Published case reports describe treatment methods extrapolated from adult MI. However, due to the rare occurrence, the most appropriate acute treatment for both MI and ventricular arrhythmia in newborn infants remains unknown...
April 2016: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/27266868/a-cluster-randomized-trial-of-a-transition-intervention-for-adolescents-with-congenital-heart-disease-rationale-and-design-of-the-chapter-2-study
#18
Andrew S Mackie, Gwen R Rempel, Adrienne H Kovacs, Miriam Kaufman, Kathryn N Rankin, Ahlexxi Jelen, Cedric Manlhiot, Samantha J Anthony, Joyce Magill-Evans, David Nicholas, Renee Sananes, Erwin Oechslin, Dimi Dragieva, Sonila Mustafa, Elina Williams, Michelle Schuh, Brian W McCrindle
BACKGROUND: The population of adolescents and young adults with congenital heart disease (CHD) is growing exponentially. These survivors are at risk of late cardiac complications and require lifelong cardiology care. However, there is a paucity of data on how to prepare adolescents to assume responsibility for their health and function within the adult health care system. Evidence-based transition strategies are required. METHODS: The Congenital Heart Adolescents Participating in Transition Evaluation Research (CHAPTER 2) Study is a two-site cluster randomized clinical trial designed to evaluate the efficacy of a nurse-led transition intervention for 16-17 year olds with moderate or complex CHD...
June 6, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27245633/regenerative-medicine-strategies-for-hypoplastic-left-heart-syndrome
#19
Josue Chery, Joshua Wong, Shan Huang, Shuyun Wang, Ming-Sing Si
Hypoplastic left heart syndrome (HLHS), the most severe and common form of single ventricle congenital heart lesions, is characterized by hypoplasia of the mitral valve, left ventricle (LV), and all LV outflow structures. While advances in surgical technique and medical management have allowed survival into adulthood, HLHS patients have severe morbidities, decreased quality of life, and a shortened lifespan. The single right ventricle (RV) is especially prone to early failure because of its vulnerability to chronic pressure overload, a mode of failure distinct from ischemic cardiomyopathy encountered in acquired heart disease...
December 2016: Tissue Engineering. Part B, Reviews
https://www.readbyqxmd.com/read/27234354/extra-cardiac-manifestations-of-adult-congenital-heart-disease
#20
REVIEW
Stephen A Gaeta, Cary Ward, Richard A Krasuski
Advancement in correction or palliation of congenital cardiac lesions has greatly improved the lifespan of congenital heart disease patients, resulting in a rapidly growing adult congenital heart disease (ACHD) population. As this group has increased in number and age, emerging science has highlighted the systemic nature of ACHD. Providers caring for these patients are tasked with long-term management of multiple neurologic, pulmonary, hepatic, renal, and endocrine manifestations that arise as syndromic associations with congenital heart defects or as sequelae of primary structural or hemodynamic abnormalities...
October 2016: Trends in Cardiovascular Medicine
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