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https://www.readbyqxmd.com/read/29028634/establishing-a-successful-transition-care-plan-for-the-adolescent-with-congenital-heart-disease
#1
Christopher Talluto
PURPOSE OF REVIEW: In this review we explore the aspects needed to develop a successful transition program for adolescents with congenital heart disease and the barriers that exist to the development of such a program. RECENT FINDINGS: We review the literature including more recent publications which support the development of a transition program in order to facilitate transfer from the pediatric to the adult healthcare system. SUMMARY: With the development of a successful transition program patients will hopefully receive high-quality, continuous life-long care in the appropriate setting...
October 11, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/29024133/plasma-natriuretic-peptide-levels-in-fetuses-with-congenital-heart-defect-and-arrhythmia-a-single-center-prospective-study
#2
Takekazu Miyoshi, Takashi Umekawa, Hiroshi Hosoda, Takashi Asada, Akihiro Fujiwara, Ken-Ichi Kurosaki, Isao Shiraishi, Michikazu Nakai, Kunihiro Nishimura, Mikiya Miyazato, Kenji Kangawa, Tomoaki Ikeda, Jun Yoshimatsu, Naoto Minamino
OBJECTIVES: Diagnosing fetal heart failure remains challenging because it is difficult to know how well the fetal myocardium will perform as loading conditions change. In adult cardiology, natriuretic peptides (NPs) are established marker of heart failure. However, the number of studies investigating NP levels in fetuses is quite limited. The aim of this study was to evaluate the significance of plasma NP levels in the assessment of heart failure in fetuses with congenital heart defect (CHD) and arrhythmia...
October 11, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29016556/the-current-status-and-future-of-cardiac-stem-progenitor-cell-therapy-for-congenital-heart-defects-from-diabetic-pregnancy
#3
REVIEW
Jianxiang Zhong, Shengbing Wang, Wei-Bin Shen, Sunjay Kaushal, Peixin Yang
Pregestational maternal diabetes induces congenital heart defects (CHDs). Cardiac dysfunction after palliative surgical procedures contributes to the high mortality of CHD patients. Autologous or allogeneic stem cell therapies are effective for improving cardiac function in animal models and clinical trials. c-kit(+) cardiac progenitor cells (CPCs), the most recognized CPCs, have the following basic properties of stem cells: self-renewal, multicellular clone formation, and differentiation into multiple cardiac lineages...
October 10, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28993401/diagnosis-and-management-of-noncardiac-complications-in-adults-with-congenital-heart-disease-a-scientific-statement-from-the-american-heart-association
#4
REVIEW
George K Lui, Arwa Saidi, Ami B Bhatt, Luke J Burchill, Jason F Deen, Michael G Earing, Michael Gewitz, Jonathan Ginns, Joseph D Kay, Yuli Y Kim, Adrienne H Kovacs, Eric V Krieger, Fred M Wu, Shi-Joon Yoo
Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD...
October 9, 2017: Circulation
https://www.readbyqxmd.com/read/28991016/process-of-transition-for-congenital-heart-patients-preventing-loss-to-follow-up
#5
Hajar Habibi, Yaso Emmanuel, Natali Chung
PURPOSE: The aim of this article is to provide an overview of our nurse-led transition clinic provided to congenital heart disease patients moving from pediatric into adult care setting. DESCRIPTION OF THE SERVICE: Nurse-led transition clinic was analyzed at various stages of young adult care from an early stage of 12 to 14 years to entering adult setting at 16 years or older. METHODS: Overview of current transition service for young adults being transferred from pediatric into adult services highlights the integral role of clinical nurse specialist as a coordinator of care...
November 2017: Clinical Nurse Specialist CNS
https://www.readbyqxmd.com/read/28988213/management-of-pneumatosis-intestinalis-in-children-over-the-age-of-6-months-a-conservative-approach
#6
Leel Nellihela, Mohamed Mutalib, David Thompson, Kammermeier Jochen, Manasvi Upadhyaya
BACKGROUND: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. AIM: Review of our experience of PI in children beyond the early infantile period...
October 7, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28980341/do-not-forget-the-parents-parents-concerns-during-transition-to-adult-care-for-adolescents-with-congenital-heart-disease
#7
E L Bratt, Å Burström, K Hanseus, A Rydberg, M Berghammer
BACKGROUND: Growing up with congenital heart disease (CHD) often means transfer to adult care and lifelong medical follow-up. An optimal transition process usually involves a multipart collaboration between the patient, their parents and other family members, and the healthcare providers. Taking an active role while knowing when it is time to step aside can be difficult for all the concerned parties, even the healthcare professionals. The aim of the present study therefore, was to explore parents' expectations and needs during their adolescent's transition to adult care...
October 5, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28977206/repair-of-double-orifice-left-av-valve-dolavv-with-endocardial-cushion-defect-in-adult
#8
Vivek Velayudhan Pillai, Jayakumar Karunakaran
Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results...
July 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28976338/false-positive-perfusion-ventilation-spect-study-for-pulmonary-embolism-in-a-patient-with-fontan-circulation
#9
Emmanouil Panagiotidis
Fontan circulation is the consequence of an operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. An 18-year old man with a history of congenital heart disease surgically treated with Fontan circulation, presented with pleuritic chest pain and a raised D-dimer level. Perfusion/ventilation SPECT was performed to exclude the possibility of pulmonary embolism (PE) that showed unilateral reduced perfusion of the left lung with a mismatched right upper lobe defect, suspicious of PE...
October 3, 2017: Molecular Imaging and Radionuclide Therapy
https://www.readbyqxmd.com/read/28975959/challenges-of-congenital-heart-disease-in-grown-up-patients
#10
Markus Schwerzmann, Fabienne Schwitz, Corina Thomet, Alexander Kadner, Jean-Pierre Pfammatter, Kerstin Wustmann
Nowadays, more than 90% of all children born with congenital heart disease (CHD) reach adult life. Although initially considered to be cured, the majority of them continue to need specialised follow-up because they require re-do interventions or are at increased risk of cardiovascular complications and premature death. Arrhythmias are the most common cause of unscheduled hospital visits for grown-up CHD (GUCH) patients, accounting for one third of emergency admissions in these patients. Some GUCH patients are also at increased risk for sudden cardiac death...
October 4, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28973413/aryl-hydrocarbon-receptor-ablation-in-cardiomyocytes-protects-male-mice-from-heart-dysfunction-induced-by-nkx2-5-haploinsufficiency
#11
Qin Wang, Yunxia Fan, Hisaka Kurita, Min Jiang, Sheryl Koch, Marepalli B Rao, Jack Rubinstein, Alvaro Puga
Epidemiological studies in humans and research in vertebrates indicates that developmental exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), a ubiquitous and biopersistent environmental toxicant, is associated with incidence of early congenital heart disease in the embryo and later in the adult. TCDD-mediated toxicity depends on the aryl hydrocarbon receptor (AHR) but the role of the TCDD-activated AHR in cardiac function is not well defined. To characterize the mechanisms responsible for AHR-mediated disruption of heart function, we generated several mouse strains with cardiomyocyte-specific Ahr gene knockout...
August 14, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28971577/development-of-quality-metrics-for-ambulatory-pediatric-cardiology-transposition-of-the-great-arteries-after-arterial-switch-operation
#12
Carissa M Baker-Smith, Karina Carlson, Jose Ettedgui, Takeshi Tsuda, K Anitha Jayakumar, Matthew Park, Nikola Tede, Karen Uzark, Craig Fleishman, David Connuck, Maggie Likes, Daniel J Penny
OBJECTIVE: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO). DESIGN: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology...
October 2, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28970652/the-need-for-extracorporeal-membrane-oxygenation-in-adults-undergoing-congenital-heart-surgery-impact-and-trends-of-utilization
#13
Salvatore Aiello, Rohit S Loomba, Connor Kriz, Matthew Buelow, Saurabh Aggarwal, Rohit R Arora
INTRODUCTION: Adults with congenital heart disease (ACHD) represent a population with unique health-care needs. Many patients require cardiac surgery, with some requiring postoperative extracorporeal membrane oxygenation (ECMO). This study aimed to identify the risk factors for the need of postoperative ECMO and characterize the impact of ECMO on admission characteristics. METHODS: Data from the 2005-2012 iterations of the Nationwide Inpatient Sample were used. ACHD admissions over 18 years with a documented cardiac surgery were included...
September 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28970037/a-novel-method-for-evaluating-regional-rv-function-in-the-adult-congenital-heart-with-low-dose-ct-and-squeez-processing
#14
Francisco J Contijoch, Daniel W Groves, Zhennong Chen, Marcus Y Chen, Elliot R McVeigh
BACKGROUND: Measuring local RV function in adult congenital heart disease (ACHD) with echocardiography or MRI is challenging because of the complex geometry and existing pacing devices. Visual assessment of ventricular function via low-dose cardiac CT has been recently performed. This pilot study assessed whether low-dose 4D cine CT combined with automatic measurement of regional shortening could quantify right-ventricular function in ACHD patients. METHODS: Seven patients with Tetralogy of Fallot either contraindicated for MRI or assessed for coronary artery disease and seven non-congenital patients were imaged with ECG-gated cardiac CT utilizing a 320-detector row scanner...
September 29, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28969764/the-role-of-pulmonary-scintigraphy-in-the-evaluation-of-adults-with-congenital-heart-disease
#15
REVIEW
Malak Itani, Manuela Matesan, Jitesh Ahuja, Mohamed Bermo, Asma S Habib, Christopher Goiney, Eric V Krieger, Hubert Vesselle
Adults with congenital heart disease represent a growing population with challenging and complex medical management. Pulmonary scintigraphy can play a valuable role in the evaluation and care of this patient population. We present a review of the variety of clinical scenarios where pulmonary scintigraphy can be helpful in the evaluation of adults with congenital heart disease, along with technical considerations associated with these studies.
November 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/28969186/adult-survival-in-ellis-van-creveld-syndrome-with-common-atrium-a-rare-case-report
#16
Mugula Sudhakar Rao, Devavrata Sahu, Hashir Kareem, Tom Devasia, Kishor Kumar Shetty
Ellis-van Creveld syndrome is a rare genetic disorder, characterized by chondral dysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. Patients with this syndrome rarely survive into adulthood. The syndrome has also been rarely reported in the Indian population. We present the case of a 56 year old female who presented with post-menopausal bleeding, and was diagnosed with Ellis van Creveld syndrome, with a common atrium and common atrioventricular valve.
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28967605/-22q11-2%C3%A2-microdeletion-syndrome-analysis-of-the-care-pathway-before-the-genetic-diagnosis
#17
T Ingrao, L Lambert, M Valduga, G Bosser, E Albuisson, B Leheup
BACKGROUND: 22q11.2 deletion syndrome (22q11.2DS) is a very broad phenotypic spectrum disorder. It can affect many organs or systems. 22q11.2DS is the most common microdeletion syndrome in humans, with a prevalence ranging from one in every 2000 to one in 4000 newborns. It seems to be more prevalent than reported and under-recognized or undiagnosed because of its inherent clinical variability and heterogeneity. In France, 15,000 patients may be affected by this disease, more than half without knowing it...
September 26, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28966315/prognostic-value-of-pulmonary-artery-compliance-in-patients-with-pulmonary-arterial-hypertension-associated-with-adult-congenital-heart-disease
#18
Xiao-Ling Cheng, Zhi-Hong Liu, Qing Gu, Xin-Hai Ni, Qin Luo, Zhi-Hui Zhao, Jian-Guo He, Chang-Ming Xiong
In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients ofAPAH-CHD who underwent a comprehensive clinical evaluation were included in this study. All patients were followed up in a 6-month interval and the primary end point was all cause of death...
September 30, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28957538/origins-and-consequences-of-congenital-heart-defects-affecting-the-right-ventricle
#19
Odilia I Woudstra, Suchit Ahuja, Jouke P Bokma, Berto J Bouma, Barbara J M Mulder, Vincent M Christoffels
Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28954835/retrospective-uk-multicentre-study-of-the-pregnancy-outcomes-of-women-with-a-fontan-repair
#20
Matthew Cauldwell, Philip J Steer, Samantha Bonner, Omar Asghar, Lorna Swan, Kenneth Hodson, Catherine E G Head, Adam Daniel Jakes, Nicola Walker, Margaret Simpson, Aidan P Bolger, Farah Siddiqui, Katherine M English, Lucy Maudlin, Dilip Abraham, Andrew J Sands, Aarthi R Mohan, Stephanie L Curtis, Louise Coats, Mark R Johnson
BACKGROUND: The population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK. METHODS: A retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK. RESULTS: 50 women had 124 pregnancies, resulting in 68 (54...
September 27, 2017: Heart: Official Journal of the British Cardiac Society
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