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Adult congenital heart disease

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https://www.readbyqxmd.com/read/28709919/morbidity-after-cardiac-surgery-in-patients-with-adult-congenital-heart-disease-in-comparison-with-acquired-disease
#1
Dimos Karangelis, Amine Mazine, Sreekanth Narsupalli, Shamarli Mendis, Gruschen Veldtman, Nicolas Nikolaidis
BACKGROUND: Due to the advancements in congenital cardiac surgery and interventional cardiology in the last five decades, more than 85% of congenital heart patients now survive to adulthood. METHODS: This retrospective study included 135 Adult Congenital Heart Disease (ACHD) patients, who had cardiac surgery at Southampton General Hospital over three consecutive years. We also included 42 patients with a structurally normal heart who had cardiac surgery for acquired cardiac conditions as a control group...
June 28, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28707365/use-of-3-d-digital-subtraction-rotational-angiography-during-cardiac-catheterization-of-infants-and-adults-with-congenital-heart-diseases
#2
Sushitha Surendran, B Rush Waller, Lucas Elijovich, Vijaykumar Agrawal, Andrew Kuhls-Gilcrist, Jason Johnson, Thomas Fagan, Shyam K Sathanandam
OBJECTIVE: To compare image quality, radiation and contrast doses required to obtain 3D-Digital subtraction rotational angiography (3D-DSRA) with 3D-Digital rotational angiography (3D-DRA) in infants (children ≤ 2 years of age) and adults with congenital heart diseases (ACHD). BACKGROUND: 3D-DRA can be performed with radiation doses comparable to bi-plane cine-angiography. However, 3D-DRA in infants requires a large contrast volume. The resolution of 3D-DRA performed in ACHD patients is limited by their soft tissue density...
July 14, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28706735/the-importance-of-copy-number-variation-in-congenital-heart-disease
#3
Gregory Costain, Candice K Silversides, Anne S Bassett
Congenital heart disease (CHD) is the most common class of major malformations in humans. The historical association with large chromosomal abnormalities foreshadowed the role of submicroscopic rare copy number variations (CNVs) as important genetic causes of CHD. Recent studies have provided robust evidence for these structural variants as genome-wide contributors to all forms of CHD, including CHD that appears isolated without extra-cardiac features. Overall, a CNV-related molecular diagnosis can be made in up to one in eight patients with CHD...
September 14, 2016: NPJ Genomic Medicine
https://www.readbyqxmd.com/read/28706585/atrial-tachyarrhythmia-in-adult-congenital-heart-disease
#4
REVIEW
Arsha Karbassi, Krishnakumar Nair, Louise Harris, Rachel M Wald, S Lucy Roche
The adult congenital heart disease (ACHD) population continues to grow and most cardiologists, emergency room physicians and family doctors will intermittently come into contact with these patients. Oftentimes this may be in the setting of a presentation with atrial tachyarrhythmia; one of the commonest late complications of ACHD and problem with potentially serious implications. Providing appropriate initial care and ongoing management of atrial tachyarrhythmia in ACHD patients requires a degree of specialist knowledge and an awareness of certain key issues...
June 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28705803/clinical-practice-guidelines-for-the-care-of-girls-and-women-with-turner-syndrome-proceedings-from-the-2016-cincinnati-international-turner-syndrome-meeting
#5
Claus H Gravholt, Niels H Andersen, Gerard S Conway, Olaf M Dekkers, Mitchell E Geffner, Karen O Klein, Angela E Lin, Nelly Mauras, Charmian A Quigley, Karen Rubin, David E Sandberg, Theo C J Sas, Michael Silberbach, Viveca Söderström-Anttila, Kirstine Stochholm, Janielle A van Alfen-van derVelden, Joachim Woelfle, Philippe F Backeljauw
Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016...
September 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28705603/ischemic-heart-disease-in-children-and-young-adults-with-congenital-heart-disease-in-sweden
#6
Maria Fedchenko, Zacharias Mandalenakis, Annika Rosengren, Georg Lappas, Peter Eriksson, Kristofer Skoglund, Mikael Dellborg
BACKGROUND: An increasing proportion of congenital heart disease (CoHD) patients survive to an age associated with increased risk of developing ischemic heart disease (IHD). The aim was to investigate the risk of developing IHD among children and young adults with CoHD. METHODS: Using the Swedish National Patient Register, we created a cohort of all CoHD patients born between January 1970 and December 1993. Ten controls matched for age, sex, county were randomly selected from the general population for each patient (n=219,816)...
July 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28704916/three-dimensional-echocardiography-in-adult-congenital-heart-disease
#7
REVIEW
Hyun Suk Yang
Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28702947/initial-validation-of-a-healthcare-needs-scale-for-youth-with-congenital-heart-disease
#8
Chi-Wen Chen, Ciao-Lin Ho, Wen-Jen Su, Jou-Kou Wang, Hung-Tao Chung, Pi-Chang Lee, Chun-Wei Lu, Be-Tau Hwang
AIM: To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. BACKGROUND: As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. DESIGN: A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease...
July 12, 2017: Journal of Advanced Nursing
https://www.readbyqxmd.com/read/28698799/adults-with-repaired-tetralogy-low-mortality-but-high-morbidity-up-to-middle-age
#9
Mark Dennis, Ben Moore, Irina Kotchetkova, Lynne Pressley, Rachael Cordina, David S Celermajer
OBJECTIVE: Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service. METHODS: One hundred and sixty-eight consecutive patients with 'simple rToF', aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included...
2017: Open Heart
https://www.readbyqxmd.com/read/28696875/status-and-challenges-of-care-in-africa-for-adults-with-congenital-heart-defects
#10
Frank Edwin, Liesl Zühlke, Heba Farouk, Ana Olga Mocumbi, Kow Entsua-Mensah, Desrie Delsol-Gyan, Fidelia Bode-Thomas, Andre Brooks, Blanche Cupido, Mark Tettey, Ernest Aniteye, Martin M Tamatey, Kofi B Gyan, Jacques Cabral Tantchou Tchoumi, Mohamed-Adel Elgamal
The 54 countries in Africa have an estimated total annual congenital heart defect (CHD) birth prevalence of 300,486 cases. More than half (51.4%) of the continental birth prevalence occurs in only seven countries. Congenital heart disease remains primarily a pediatric health issue in Africa because of the deficient health-care systems: the adults with CHD made up just 10% of patients with CHD in Ghana, and 13.7% of patients with CHD presenting for surgery in Mozambique. With Africa's population projected to double in the next 35 years, the already deficient health systems for CHD care will suffer unbearable strain unless determined and courageous action is undertaken by the African leaders...
July 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28696220/prevention-of-sudden-cardiac-death-in-adults-with-congenital-heart-disease-do-the-guidelines-fall-short
#11
Jim T Vehmeijer, Zeliha Koyak, Werner Budts, Louise Harris, Candice K Silversides, Erwin N Oechslin, Berto J Bouma, Aeilko H Zwinderman, Barbara J M Mulder, Joris R de Groot
BACKGROUND: Sudden cardiac death (SCD) is a major cause of mortality in adult congenital heart disease (ACHD) patients. SCD may be prevented by implantable cardioverter-defibrillator (ICD) implantation, but patient stratification remains troublesome. The 2014 Consensus Statement on Arrhythmias in ACHD patients and the 2015 European Society of Cardiology Guidelines specified recommendations for ICD implantation in ACHD patients for the first time. We assess the discriminative ability of these ICD recommendations for SCD in ACHD patients...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28690220/-investigation-and-analysis-for-current-situation-and-pathogenesis-relevant-to-pulmonary-hypertension
#12
Si Lei, Doudou Tang, Nianru Xu, Shangjie Wu
To investigate the demographic characteristics and the causes for pulmonary hypertension (PH) in adult patients.
 Methods: A total of 2 508 adult patients diagnosed as PH, who came from the Second Xiangya Hospital of Central South University from January 2010 to December 2014, were retrospectively investigated. All subjects underwent the clinical diagnosis, or the echocardiographic diagnosis, or thetraditional hemodynamic criteria by right heart catheterization (RHC). The patient's data including hospital numbers, gender, ages, primary diseases, etc, are collected and analyzed...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28687271/number-of-thoracotomies-predicts-impairment-in-lung-function-and-exercise-capacity-in-patients-with-congenital-heart-disease
#13
Jan Müller, Peter Ewert, Alfred Hager
OBJECTIVE: Many patients with congenital heart disease (CHD) require surgery to ensure survival into adulthood. But history of previous thoracotomies is associated with respiratory muscle weakness, impairments in chest wall compliance, and moderately to severely impaired lung function. This study evaluated the impact of thoracotomies on functional outcome in patients with CHD. PATIENTS AND METHODS: In total 1372 adolescents and adults with CHD (32.4±11.5 years, 624 female), who underwent spirometry and cardiopulmonary exercise testing in our institution from January 2010 to August 2015, were analyzed...
July 4, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28683534/a-case-of-alagille-syndrome-presenting-with-chronic-cholestasis-in-an-adult
#14
Jihye Kim, Bumhee Yang, Namyoung Paik, Yon Ho Choe, Yong-Han Paik
Alagille syndrome (AGS) is a complex multisystem disorder that involves mainly the liver, heart, eyes, face, and skeleton. The main associated clinical features are chronic cholestasis due to a paucity of intrahepatic bile ducts, congenital heart disease primarily affecting pulmonary arteries, vertebral abnormalities, ocular embryotoxon, and peculiar facies. The manifestations generally become evident at a pediatric age. AGS is caused by defects in the Notch signaling pathway due to mutations in JAG1 or NOTCH2...
July 7, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28681101/clinical-implications-of-eicosapentaenoic-acid-arachidonic-acid-ratio-epa-aa-in-adult-patients-with-congenital-heart-disease
#15
Miki Kanoh, Kei Inai, Tokuko Shinohara, Hirofumi Tomimatsu, Toshio Nakanishi
Recent studies showed that a low ratio between the levels of eicosapentaenoic acid and those of arachidonic acid (EPA/AA) is associated with higher incidence of coronary artery disease and poor prognosis of heart failure, arrhythmia, and cardiac sudden death. However, the clinical implications of EPA/AA in adult patients with congenital heart disease remain unclear. We aimed to assess the prognostic value of EPA/AA regarding cardiac events in adult patients with congenital heart disease. We measured the serum levels of eicosapentaenoic acid and arachidonic acid in 130 adult patients (median age, 31 years) stratified into two groups according to their EPA/AA (low, ≤0...
July 5, 2017: Heart and Vessels
https://www.readbyqxmd.com/read/28678047/imaging-of-the-pulmonary-valve-in-the-adults
#16
Ricardo H Pignatelli, Cory Noel, S Chandra B Reddy
PURPOSE OF REVIEW: Pulmonary valve is the least imaged of the cardiac valves in adults. This review will address the strengths and the limitations of various imaging modalities that are commonly used for evaluation of pulmonary valve diseases in the adults. RECENT FINDINGS: Valvular pulmonary stenosis is mostly congenital and pulmonary regurgitation is usually an acquired pulmonary valve disease. Combined pulmonary stenosis and pulmonary regurgitation as sequel to previous surgeries for congenital heart diseases is the most common form of pulmonary valve disease in the adults...
July 3, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28674885/red-blood-cells-in-clinical-proteomics
#17
Ana Sofia Carvalho, Manuel S Rodriguez, Rune Matthiesen
Red blood cells (RBCs) are known for their role in oxygen and carbon dioxide transport. The main function of RBCs is directly linked to many diseases that cause low oxygen levels in tissues such as congenital heart disease in adults, chronic obstructive pulmonary disease, sleep apnea, sickle cell disease, etc. Red blood cells are a direct target for a number of parasitic diseases such as malaria (Plasmodium) and similar parasites of the phylum Apicomplexa (Toxoplasma, Theileria, Eimeria, Babesia, and Cryptosporidium)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28674625/gender-related-differences-in-self-reported-dental-care-in-adults-with-congenital-heart-disease-at-increased-risk-of-infective-endocarditis
#18
Susann Schmidt, Marlies Ramseier-Hadorn, Corina Thomet, Kerstin Wustmann, Markus Schwerzmann
OBJECTIVE: Adults with congenital heart disease (CHD) are at increased risk of infective endocarditis (IE). Women with CHD have a lower IE risk, potentially due to gender-related differences in dental care. We aimed to assess self-reported dental hygiene measures in adults with CHD, and to identify factors associated with good oral hygiene. METHODS AND RESULTS: Descriptive study includes 187 adults with CHD at increased risk of IE. The patients' IE knowledge was assessed using an adapted version of the Leuven Knowledge Questionnaire for CHD...
2017: Open Heart
https://www.readbyqxmd.com/read/28671804/valvular-heart-disease-in-adults-etiologies-classification-and-diagnosis
#19
Steven M Hollenberg
The prevalence of valvular heart disease (VHD) in the United States was estimated to be approximately 2.5% in the 1990s. The prevalence currently is thought to be increasing because of more accurate diagnostic methods and aging of the population. Mitral regurgitation (MR) is the most common valve defect, followed by aortic stenosis (AS) and aortic regurgitation (AR). Degenerative disease is the most common etiology of MR, AS, and AR, though these forms of VHD also can be caused by congenital valve defects, systemic inflammatory diseases, endocarditis, and many other conditions...
June 2017: FP Essentials
https://www.readbyqxmd.com/read/28669511/illness-perceptions-in-adult-congenital-heart-disease-a-multi-center-international-study
#20
Jessica Rassart, Silke Apers, Adrienne H Kovacs, Philip Moons, Corina Thomet, Werner Budts, Junko Enomoto, Maayke A Sluman, Jou-Kou Wang, Jamie L Jackson, Paul Khairy, Stephen C Cook, Raghavan Subramanyan, Luis Alday, Katrine Eriksen, Mikael Dellborg, Malin Berghammer, Bengt Johansson, Gwen R Rempel, Samuel Menahem, Maryanne Caruana, Gruschen Veldtman, Alexandra Soufi, Susan M Fernandes, Kamila S White, Edward Callus, Shelby Kutty, Koen Luyckx
BACKGROUND: Illness perceptions are cognitive frameworks that patients construct to make sense of their illness. Although the importance of these perceptions has been demonstrated in other chronic illness populations, few studies have focused on the illness perceptions of adults with congenital heart disease (CHD). This study examined (1) inter-country variation in illness perceptions, (2) associations between patient characteristics and illness perceptions, and (3) associations between illness perceptions and patient-reported outcomes...
June 26, 2017: International Journal of Cardiology
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