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Adult congenital heart disease, structural heart disease

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https://www.readbyqxmd.com/read/28342658/educational-needs-of-adolescents-with-congenital-heart-disease-impact-of-a-transition-intervention-programme
#1
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/sudden-death-in-athletes
#2
Domenico Corrado, Alessandro Zorzi
Competitive sports activity is associated with an increased risk of sudden cardiovascular death (SCD) in adolescents and young adults with clinically silent cardiovascular disorders. While in middle-aged/senior athletes atherosclerotic coronary artery disease accounts for the vast majority of SCDs, in young athletes the spectrum of substrates is wider and includes inherited (cardiomyopathies) and congenital (anomalous origin of coronary arteries) structural heart diseases. Inherited ion channel diseases have been implicated in SCDs occurring with an apparently normal heart at autopsy...
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
#3
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
#4
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
#5
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
#6
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...
March 14, 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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
January 2017: 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
#19
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
#20
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
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