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Adult congenital heart disease, structural heart disease

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https://www.readbyqxmd.com/read/28548989/imaging-adult-patients-with-fontan-circulation
#1
Salil Ginde, Benjamin H Goot, Peter C Frommelt
PURPOSE OF REVIEW: Survival after the Fontan procedure for palliation of single ventricle congenital heart disease has improved. However, adults with Fontan circulation are at risk for several complications including heart failure, thromboembolism, and protein-losing enteropathy. This review discusses the role of noninvasive imaging for surveillance and early detection of anatomic and functional abnormalities of the Fontan circulation that can impact the risk for Fontan failure over time...
May 25, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28493591/transcatheter-interventions-in-adults-with-congenital-heart-disease-surveys-from-the-society-for-cardiovascular-angiography-and-interventions-to-identify-current-patterns-of-care-and-perception-on-training-requirements
#2
Subeer K Wadia, Michael J Accavitti, Gareth J Morgan, Damien Kenny, Ziyad M Hijazi, Thomas K Jones, Allison K Cabalka, Doff B McElhinney, Clifford J Kavinsky
BACKGROUND: Interventional catheterization is central to the care of Adults with Congenital Heart Disease (ACHD). Current standards for care provision and training in ACHD intervention are lacking. We sought to examine trends in current practice and training amongst interventionalists. METHODS: We analyzed the results of two separate international surveys in June 2016. One was sent to all active members from the Society of Cardiovascular Angiography and Interventions (SCAI) who self-identified Structural Heart Disease or Congenital Heart Disease as a practice area...
May 11, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28477319/transition-of-care-in-congenital-heart-disease-ensuring-the-proper-handoff
#3
REVIEW
Angela Lee, Barbara Bailey, Geraldine Cullen-Dean, Sandra Aiello, Joanne Morin, Erwin Oechslin
BACKGROUND: With great advances in medical and surgical care, most congenital heart disease patients are living in to adulthood and require lifelong surveillance and expert care for adult onset complications. Care lapse and lack of successful transfer from pediatric to adult care put young adults at risk for increased morbidity and premature death. Hence, transition and transfer from pediatric to adult care is a crucial and critical process to provide access to specialized care and lifelong surveillance...
June 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28420661/person-centred-transition-programme-to-empower-adolescents-with-congenital-heart-disease-in-the-transition-to-adulthood-a-study-protocol-for-a-hybrid-randomised-controlled-trial-stepstones-project
#4
Mariela Acuña Mora, Carina Sparud-Lundin, Ewa-Lena Bratt, Philip Moons
INTRODUCTION: When a young person grows up, they evolve from an independent child to an empowered adult. If an individual has a chronic condition, this additional burden may hamper adequate development and independence. Transition programmes for young persons with chronic disorders aim to provide the necessary skills for self-management and participation in care. However, strong evidence on the effects of these interventions is lacking. Therefore, as part of the STEPSTONES project (Swedish Transition Effects Project Supporting Teenagers with chrONic mEdical conditionS), we propose a trial to assess the effectiveness of a structured, person-centred transition programme to empower adolescents with congenital heart disease in the transition to adulthood...
April 17, 2017: BMJ Open
https://www.readbyqxmd.com/read/28402450/sudden-cardiac-death-in-adults-with-congenital-heart-disease-does-qrs-complex-fragmentation-discriminate-in-structurally-abnormal-hearts
#5
Jim T Vehmeijer, Zeliha Koyak, Jouke P Bokma, Werner Budts, Louise Harris, Barbara J M Mulder, Joris R de Groot
Aims: Sudden cardiac death (SCD) causes a large portion of all mortality in adult congenital heart disease (ACHD) patients. However, identification of high-risk patients remains challenging. Fragmented QRS-complexes (fQRS) are a marker for SCD in patients with acquired heart disease but data in ACHD patients are lacking. We therefore aim to evaluate the prognostic value of fQRS for SCD in ACHD patients. Methods and results: From a multicentre cohort of 25 790 ACHD patients, we included tachyarrhythmic SCD cases (n = 147), and controls (n = 266) matched by age, gender, congenital defect and (surgical) intervention...
April 10, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28342658/educational-needs-of-adolescents-with-congenital-heart-disease-impact-of-a-transition-intervention-programme
#6
Magalie Ladouceur, Johanna Calderon, Maladon Traore, Radhia Cheurfi, Christine Pagnon, Diala Khraiche, Fanny Bajolle, Damien Bonnet
BACKGROUND: Adolescents and young adults with congenital heart disease (CHD) have complex health needs and require lifelong follow-up. Interventions to facilitate the paediatric-to-adult healthcare transition are recommended, but outcomes remain largely under-investigated. AIMS: To identify the educational needs and the impact of a transition intervention on knowledge and self-management skills in adolescents and young adults with CHD. METHODS: From September 2014 to May 2015, 115 adolescents and young adults with CHD (mean age 17±2 years; 47 girls) were consecutively enrolled...
March 22, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28318658/temporary-removal-sudden-death-in-athletes
#7
Domenico Corrado, Alessandro Zorzi
The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.
March 10, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28302746/current-interventional-and-surgical-management-of-congenital-heart-disease-specific-focus-on-valvular-disease-and-cardiac-arrhythmias
#8
REVIEW
Kimberly A Holst, Sameh M Said, Timothy J Nelson, Bryan C Cannon, Joseph A Dearani
Successful outcome in the care of patients with congenital heart disease depends on a comprehensive multidisciplinary team. Surgery is offered for almost every heart defect, despite complexity. Early mortality for cardiac surgery in the neonatal period is ≈10% and beyond infancy is <5%, with 90% to 95% of patients surviving with a good quality of life into the adult years. Advances in imaging have facilitated accurate diagnosis and planning of interventions and surgical procedures. Similarly, advances in the perioperative medical management of patients, particularly with intensive care, has also contributed to improving outcomes...
March 17, 2017: Circulation Research
https://www.readbyqxmd.com/read/28302745/current-status-and-future-potential-of-transcatheter-interventions-in-congenital-heart-disease
#9
REVIEW
Damien P Kenny, Ziyad M Hijazi
Percutaneous therapies for congenital heart disease have evolved rapidly in the past 3 decades. This has occurred despite limited investment from industry and support from regulatory bodies resulting in a lack of specific device development. Indeed, many devices remain off-label with a best-fit approach often required, spurning an innovative culture within the subspecialty, which had arguably laid the foundation for many of the current and evolving structural heart interventions. Challenges remain, not least encouraging device design focused on smaller infants and the inevitable consequences of somatic growth...
March 17, 2017: Circulation Research
https://www.readbyqxmd.com/read/28302744/noninvasive-imaging-in-adult-congenital-heart-disease
#10
REVIEW
Luke J Burchill, Jennifer Huang, Justin T Tretter, Abigail M Khan, Andrew M Crean, Gruschen R Veldtman, Sanjiv Kaul, Craig S Broberg
Multimodality cardiovascular imaging plays a central role in caring for patients with congenital heart disease (CHD). CHD clinicians and scientists are interested not only in cardiac morphology but also in the maladaptive ventricular responses and extracellular changes predisposing to adverse outcomes in this population. Expertise in the applications, strengths, and pitfalls of these cardiovascular imaging techniques as they relate to CHD is essential. The purpose of this article is to provide an overview of cardiovascular imaging in CHD...
March 17, 2017: Circulation Research
https://www.readbyqxmd.com/read/28302382/congenital-heart-defect-causing-mutation-in-nkx2-5-displays-in-vivo-functional-deficit
#11
Abeer F Zakariyah, Rashida F Rajgara, John P Veinot, Ilona S Skerjanc, Patrick G Burgon
The Nkx2.5 gene encodes a transcription factor that plays a critical role in heart development. In humans, heterozygous mutations in NKX2.5 result in congenital heart defects (CHDs). However, the molecular mechanisms by which these mutations cause the disease remain unknown. NKX2.5-R142C is a mutation that was reported to be associated with atrial septal defect (ASD) and atrioventricular (AV) block in 13-patients from one family. The R142C mutation is located within both the DNA-binding domain and the nuclear localization sequence of NKX2...
April 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28291620/pacing-in-congenital-heart-disease-a-four-decade-experience-in-a-single-tertiary-centre
#12
Disha Midha, Zhong Chen, David G Jones, Howell J Williams, Karen Lascelles, Julian Jarman, Jonathan Clague, Janice Till, Konstatinos Dimopoulos, Sonya V Babu-Narayan, Vias Markides, Michael A Gatzoulis, Tom Wong
BACKGROUND: The increased risk of brady- and tachy-arrhythmias in the congenital heart disease (CHD) population means that cardiac rhythm management devices are often required at an early age and expose patients to device-related complications. The present study drew upon four decades of experience at a tertiary adult congenital heart disease ACHD center and aimed to investigate the indication for cardiac implantable electronic devices (CIEDs) and predictors of late device-related complication requiring re-intervention...
March 6, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28185475/4d-flow-magnetic-resonance-imaging-role-in-pediatric-congenital-heart-disease
#13
Claire M Lawley, Kathryn M Broadhouse, Fraser M Callaghan, David S Winlaw, Gemma A Figtree, Stuart M Grieve
Imaging-based evaluation of cardiac structure and function remains paramount in the diagnosis and monitoring of congenital heart disease in childhood. Accurate measurements of intra- and extracardiac hemodynamics are required to inform decision making, allowing planned timing of interventions prior to deterioration of cardiac function. Four-dimensional flow magnetic resonance imaging is a nonionizing noninvasive technology that allows accurate and reproducible delineation of blood flow at any anatomical location within the imaging volume of interest, and also permits derivation of physiological parameters such as kinetic energy and wall shear stress...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28122228/temporally-distinct-six2-positive-second-heart-field-progenitors-regulate-mammalian-heart-development-and-disease
#14
Zhengfang Zhou, Jingying Wang, Chaoshe Guo, Weiting Chang, Jian Zhuang, Ping Zhu, Xue Li
The embryonic process of forming a complex structure such as the heart remains poorly understood. Here, we show that Six2 marks a dynamic subset of second heart field progenitors. Six2-positive (Six2(+)) progenitors are rapidly recruited and assigned, and their descendants are allocated successively to regions of the heart from the right ventricle (RV) to the pulmonary trunk. Global ablation of Six2(+) progenitors resulted in RV hypoplasia and pulmonary atresia. An early stage-specific ablation of a small subset of Six2(+) progenitors did not cause any apparent structural defect at birth but rather resulted in adult-onset cardiac hypertrophy and dysfunction...
January 24, 2017: Cell Reports
https://www.readbyqxmd.com/read/28062939/early-transverse-tubule-development-begins-in-utero-in-the-sheep-heart
#15
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
#16
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
#17
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
#18
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-5-year-experience
#19
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 are found. Similar clinics for children have proven beneficial; the benefit of a HMC in an adult population has been difficult to determine. AIM: To review the clinical demographics and echocardiographic information of patients presenting to our HMC, to assess what proportion of significance structural heart disease had and determine the common structural abnormalities in this population...
February 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/27836946/heart-transplantation-in-adult-congenital-heart-disease
#20
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
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