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Adult congenital heart disease

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https://www.readbyqxmd.com/read/29457362/a-multinational-observational-investigation-of-illness-perceptions-and-quality-of-life-among-patients-with-a-fontan-circulation
#1
Christina E Holbein, Nicholas D Fogleman, Kevin Hommel, Silke Apers, Jessica Rassart, Philip Moons, Koen Luyckx, Maayke A Sluman, Junko Enomoto, Bengt Johansson, Hsiao-Ling Yang, Mikael Dellborg, Raghavan Subramanyan, Jamie L Jackson, Werner Budts, Adrienne H Kovacs, Stacey Morrison, Martha Tomlin, Kathy Gosney, Alexandra Soufi, Katrine Eriksen, Corina Thomet, Malin Berghammer, Luis Alday, Edward Callus, Susan M Fernandes, Maryanne Caruana, Samuel Menahem, Stephen C Cook, Gwen R Rempel, Kamila White, Paul Khairy, Shelby Kutty, Gruschen Veldtman
OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents...
February 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29457357/improving-outpatient-advance-care-planning-for-adults-with-congenital-or-pediatric-heart-disease-followed-in-a-pediatric-heart-failure-and-transplant-clinic
#2
Lindsay A Edwards, Christine Bui, Antonio G Cabrera, Jill Ann Jarrell
OBJECTIVE: To improve outpatient advanced care planning (ACP) for adults with congenital/pediatric heart disease followed in a pediatric heart failure (HF) and transplant clinic through quality improvement (QI) methodology. DESIGN: A one-year QI project was completed. We conducted quarterly chart reviews and incorporated feedback from the providers to direct subsequent interventions. PATIENTS AND SETTING: Patients ≥18 years of age seen in the HF and Transplant Clinic for follow-up visit were included in analysis...
February 18, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29456231/late-onset-choreoathetotic-syndrome-following-heart-surgery-in-adults-with-end-stage-renal-disease
#3
Mohamed Amine Hamzi, Kawtar Hassani, Driss El Kabbaj
Choreoathetotic syndrome is a rare complication of open cardiac surgery that is seen usually in children after surgery for congenital cardiac anomalies. Here, we report two cases of adult patients with end-stage renal disease (ESRD) on regular hemodialysis who developed acute choreoathetotic syndrome few days after cardiac surgeries under cardiopulmonary bypass (CPB). Improvement was seen after an interval with complete resolution in one case. Investigations of the cause have been noncontributory. Long CPB time seems to be the main identified risk factor in these cases...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29455367/adolescents-and-young-adults-living-with-congenital-heart-disease-coping-stress-reactivity-and-emotional-functioning
#4
Jennifer D Monti, Jamie L Jackson, Kathryn Vannatta
Living with congenital heart disease (CHD) presents survivors with numerous stressors, which may contribute to emotional problems. This study examined (a) whether coping with CHD-related stress predicts symptoms of depression and anxiety, and (b) whether associations between coping and emotional distress are moderated by involuntary stress reactivity. Adolescents and young adults diagnosed with CHD (Mage = 26.4) were recruited from pediatric and adult outpatient cardiology clinics. Participants (N = 168) completed online self-report measures...
February 17, 2018: Journal of Clinical Psychology in Medical Settings
https://www.readbyqxmd.com/read/29452367/variant-in-human-pofut1-reduces-enzymatic-activity-and-likely-causes-a-recessive-microcephaly-global-developmental-delay-with-cardiac-and-vascular-features
#5
Hideyuki Takeuchi, Derek Wong, Michael Schneider, Hudson H Freeze, Megumi Takeuchi, Steven J Berardinelli, Atsuko Ito, Hane Lee, Stanley F Nelson, Robert S Haltiwanger
Protein O-fucosyltransferase-1 (POFUT1) adds O-fucose monosaccharides to epidermal growth factor-like (EGF) repeats found on approximately 100 mammalian proteins, including Notch receptors. Haploinsufficiency of POFUT1 has been linked to adult-onset Dowling Degos Disease (DDD) with hyperpigmentation defects. Homozygous deletion of mouse Pofut1 results in embryonic lethality with severe Notch-like phenotypes including defects in somitogenesis, cardiogenesis, vasculogenesis, and neurogenesis, but the extent to which POFUT1 is required for normal human development is not yet understood...
February 14, 2018: Glycobiology
https://www.readbyqxmd.com/read/29440825/comparison-of-skin-dose-measurement-using-nanodot-%C3%A2-dosimeter-and-machine-readings-of-radiation-dose-during-cardiac-catheterization-in-children
#6
Duraisamy Balaguru, Matthew Rodriguez, Stephanie Leon, Louis K Wagner, Charles W Beasley, Andrew Sultzer, Mohammed T Numan
Objectives: Direct measurement of skin dose of radiation for children using optically stimulated luminescence (OSL) technology using nanoDot ® (Landauer, Glenwood, IL, USA). Background: Radiation dose is estimated as cumulative air kerma (AK) and dosearea product based on standards established for adult size patients. Body size of pediatric patients who undergo cardiac catheterization for congenital heart disease vary widely from newborn to adolescence. Direct, skindose measurement applying OSL technology may eliminate errors in the estimate...
January 2018: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/29440224/competent-for-commitment-you-ve-got-to-have-heart
#7
REVIEW
Rajan Jain, Jonathan A Epstein
The mature heart is composed primarily of four different cell types: cardiac myocytes, endothelium, smooth muscle, and fibroblasts. These cell types derive from pluripotent progenitors that become progressively restricted with regard to lineage potential, giving rise to multipotent cardiac progenitor cells and, ultimately, the differentiated cell types of the heart. Recent studies have begun to shed light on the defining characteristics of the intermediary cell types that exist transiently during this developmental process and the extrinsic and cell-autonomous factors that influence cardiac lineage decisions and cellular competence...
January 1, 2018: Genes & Development
https://www.readbyqxmd.com/read/29440121/risk-of-dementia-in-adults-with-congenital-heart-disease-population-based-cohort-study
#8
Carina N Bagge, Victor W Henderson, Henning B Laursen, Kasper Adelborg, Morten Olsen, Nicolas L Madsen
Background -More children with congenital heart disease (CHD) are surviving to adulthood, and CHD is associated with risk factors for dementia. We compared the risk of dementia in CHD adults to that of the general population. Methods -In this cohort study, we used medical registries and a medical record review covering all Danish hospitals to identify adults with CHD diagnosed between 1963 and 2012. These individuals with CHD were followed from January 1, 1981, 30 years of age, or date of first CHD registration (index date for matched members of the general population cohort) until hospital diagnosis of dementia, death, emigration, or end of study (December 31, 2012)...
February 12, 2018: Circulation
https://www.readbyqxmd.com/read/29439889/hematuria-in-an-adult-with-congenital-heart-disease
#9
Jennifer Noble, Shahnawaz M Amdani, Richard U Garcia, Rajan Arora
BACKGROUND: Adults with congenital heart disease (CHD) in the United States now outnumber children with CHD, due in part to the improvement in surgical and medical management. This growing population may present postoperatively to the emergency department (ED) with nonspecific complaints from unforseen complications secondary to cardiac intervention. CASE REPORT: We describe a 39-year-old male who presented to the ED with hematuria and dysuria after he underwent percutaneous device ventricular septal defect (VSD) closure 10 days before...
February 10, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29437760/transvenous-lead-extraction-in-adults-with-congenital-heart-disease-insights-from-a-20-year-single-center-experience
#10
Jean-Baptiste Gourraud, Marie-A Chaix, Azadeh Shohoudi, Pierre Pagé, Marc Dubuc, Bernard Thibault, Nancy C Poirier, Annie Dore, François Marcotte, François-Pierre Mongeon, Anita W Asgar, Réda Ibrahim, Paul Khairy, Blandine Mondésert
BACKGROUND: Safety and feasibility data on transvenous lead extraction (TLE) in the challenging population of adults with congenital heart disease (A-CHD) are limited. Herein, we report the results of TLE in A-CHD during a 20-year period. METHODS AND RESULTS: All consecutive TLE procedures in A-CHD were included in a monocentric prospective registry from 1996. A total of 121 leads were extracted in 49 A-CHD (median age, 38 years; 51% men) during 71 TLE procedures...
February 2018: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/29437602/adult-congenital-heart-disease-in-a-nationwide-population-2000-2014-epidemiological-trends-arrhythmia-and-standardized-mortality-ratio
#11
Mei-Hwan Wu, Chun-Wei Lu, Hui-Chi Chen, Feng-Yu Kao, San-Kuei Huang
BACKGROUND: The adult congenital heart disease (ACHD) population will grow with medical advances, but data are limited. We investigated the epidemiological profile of ACHD in Taiwan, a country with a congenital heart disease program since 1955, population of 23 million, and easily accessible high-standard medical care. METHODS AND RESULTS: ACHD patients, born after 1954, were identified from the nationwide database 2000-2014. The ACHD prevalence in the population aged 18 to 59 was 140...
February 8, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29437076/transcatheter-pulmonary-valve-replacement-in-patients-with-congenital-heart-disease
#12
Shantelle Bartra
Transcatheter pulmonary valve replacement is now a feasible alternative to surgical pulmonary valve replacement in children and adults with dysfunctional right ventricular outflow conduits. Currently, 2 types of valves can be used for this application. This article provides an overview of the procedure and how it is performed, indications and contraindications for transcatheter pulmonary valve replacement, and short- and long-term outcomes. Nursing considerations mainly focus on educating patients, preventing bleeding and infection, monitoring renal function, and preventing injury to the catheter insertion site...
February 2018: Critical Care Nurse
https://www.readbyqxmd.com/read/29436381/subcutaneous-treprostinil-in-congenital-heart-disease-related-pulmonary-arterial-hypertension
#13
Nika Skoro-Sajer, Christian Gerges, Olga Hajnalka Balint, Dora Kohalmi, Monika Kaldararova, Iveta Simkova, Johannes Jakowitsch, Harald Gabriel, Helmut Baumgartner, Mario Gerges, Roela Sadushi-Kolici, David S Celermajer, Irene Marthe Lang
OBJECTIVE: To assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment. METHODS: Consecutive adult patients with CHD-PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study. RESULTS: Advanced CHD-PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13)...
February 7, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29433351/transcatheter-pulmonary-valve-implantation-will-it-replace-surgical-pulmonary-valve-replacement
#14
Ahmed Kheiwa, Punag Divanji, Vaikom S Mahadevan
Right ventricular outflow tract (RVOT) dysfunction is a common hemodynamic challenge for adults with congenital heart disease (ACHD), including patients with repaired tetralogy of Fallot (TOF), truncus arteriosus (TA), and those who have undergone the Ross procedure for congenital aortic stenosis and the Rastelli repair for transposition of great vessels. Pulmonary valve replacement (PVR) has become one of the most common procedures performed for ACHD patients. Areas covered: Given the advances in transcatheter technology, we conducted a detailed review of the available studies addressing the indications for PVR, historical background, evolving technology, procedural aspects, and the future direction, with an emphasis on ACHD patients...
February 12, 2018: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/29433184/transoral-versus-transnasal-approach-for-craniovertebral-junction-pathologies-never-say-never
#15
Massimiliano Visocchi, Francesco Signorelli, Chenlong Liao, Mario Rigante, Gaetano Paludetti, Giuseppe Barbagallo, Alessandro Olivi
OBJECTIVE: For many years, the microsurgical transoral approach (TOA) has been accepted as the "gold standard" for the surgical treatment of a variety of congenital, developmental, and acquired pathologies affecting the craniovertebral junction. In the present study, we try to investigate both experimental and clinical fronts of such a challenging surgery, starting from the updated literature experience. TOA is actually presented as an "old-fashioned" surgical technique dealing with possible bacterial contamination, the need of postoperative nose gastric tube feeding for a week, the possible nasopharyngeal incompetence, and the postoperative tongue swelling...
February 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29432304/anemia-in-adolescents-and-young-adult-patients-with-congenital-heart-disease
#16
Juan L Rodríguez-Hernández, Fayna Rodríguez-González, Efrén Martínez-Quintana
INTRODUCTION: Despite anemia in acquired heart disease being a common problem, little is known in patients with congenital heart disease (CHD). METHODS: In total, 544 consecutive stable noncyanotic CHD patients were studied to determine demographic, clinical, and analytic parameters. Anemia was defined as a condition in which hemoglobin concentration was <13 g/dL in male individuals and <12 g/dL in female individuals. RESULTS: In total, 49 (9%) CHD patients had anemia...
February 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29426959/acute-effect-of-inhaled-iloprost-in-children-with-pulmonary-arterial-hypertension-associated-with-simple-congenital-heart-defects
#17
Qiangqiang Li, Konstantinos Dimopoulos, Chen Zhang, Yan Zhu, Qian Liu, Hong Gu
Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH-CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included...
February 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29425288/does-frequently-inadequate-adult-care-threaten-the-outcome-of-congenital-heart-disease-after-successful-paediatric-treatment
#18
Helmut Baumgartner
No abstract text is available yet for this article.
February 7, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29421004/variation-in-practice-patterns-in-device-closure-of-atrial-septal-defects-and-patent-ductus-arteriosus-an-analysis-of-data-from-the-improving-pediatric-and-adult-congenital-treatment-impact-registry
#19
Michael L O'Byrne, Kevin F Kennedy, Jonathan J Rome, Andrew C Glatz
Practice variation is a potentially important measure of healthcare quality. The IMPACT registry provides a representative national sample with which to study practice variation in trans-catheter interventions for congenital heart disease. METHODS: We studied cases for closure of atrial septal defect (ASD) and patent ductus arteriosus (PDA) in IMPACT between January 1, 2011, and September 30, 2015, using hierarchical multivariate models studying (1) the distribution of indications for closure and (2) in patients whose indication for closure was left (LVVO) or right ventricular volume overload (RVVO), the factors influencing probability of closure of a small defect (either in size or in terms of the magnitude of shunt)...
February 2018: American Heart Journal
https://www.readbyqxmd.com/read/29417167/maternal-no2-exposure-induces-cardiac-hypertrophy-in-male-offspring-via-ros-hif-1%C3%AE-transcriptional-regulation-and-aberrant-dna-methylation-modification-of-csx-nkx2-5
#20
Yingying Zhang, Wei Yan, Xiaotong Ji, Huifeng Yue, Guangke Li, Nan Sang
Maternal exposure to nitrogen dioxide (NO2) poses a risk for morbidity and mortality in infantile congenital heart diseases and even adult cardiovascular diseases. However, the experimental evidence supporting these effects is insufficient, and the related regulatory mechanisms are unknown. In the present study, we aimed to determine whether maternal NO2 exposure causes cardiac hypertrophy-related consequences in offspring, and if so, how these adverse effects occur in the postnatal heart. The results indicate that in mice, maternal NO2 exposure causes cardiac hypertrophy in male offspring...
February 7, 2018: Archives of Toxicology
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