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Adult congenital heart disease

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https://www.readbyqxmd.com/read/28549022/pictorial-review-of-surgical-anatomy-in-adult-congenital-heart-disease
#1
Carlo N De Cecco, Giuseppe Muscogiuri, José M Madrid Pérez, Marwen Eid, Pal Suranyi, Virginia W Lesslie, Gorka Bastarrika
The survival rate of patients with congenital heart disease (CHD) has dramatically improved over the last 2 decades because of technological and surgical advances in diagnosis and treatment, respectively. The vast majority of CHD patients are, in fact, amenable to treatment by either device closure or surgery. Considering the wide spectrum of surgical procedures and complex native and derived anatomy, continuous and detailed follow-up is of paramount importance. Cardiac magnetic resonance and cardiac computed tomography angiography are the cornerstones of diagnosis and follow-up of CHD, allowing for comprehensive noninvasive assessment of the heart, coronary tree, and intrathoracic great vessels, along with both morphological and functional evaluation...
May 25, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28548989/imaging-adult-patients-with-fontan-circulation
#2
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/28545820/extracardiac-conduit-fontan-outcome-data-in-early-adulthood
#3
Dominica Zentner, Caitlin Cheshire, Leeanne Grigg
BACKGROUND: To describe the survival and health outcome status of young adults with an extracardiac Fontan procedure performed either as a primary or conversion (secondary) Fontan surgery. METHODS: The database of the Adult Congenital Heart disease service at the Royal Melbourne Hospital was interrogated to identify all adults who had undergone a primary extracardiac conduit Fontan (n=29) or a Fontan conversion with this procedure (n=8). We then determined vital status, age, original anatomy and functional status in early adulthood in both groups...
May 3, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28544839/isolated-right-ventricular-apical-trabecular-hypoplasia-a-case-report
#4
Qi Tan, Jie Zi, Mei Zhu, Anbiao Wang
Right ventricular (RV) hypoplasia with isolated myocardial disease and complete absence of RV trabeculae is a rare congenital heart disease, the current treatment of which is ineffective. Here, a rare case is presented of a middle-aged female patient with RV hypoplasia complicated by tricuspid regurgitation, right atrial thrombus, and atrial fibrillation. The patient was treated with a one-and-a-half ventricular repair, and showed significant improvements in cardiac function and physical activity tolerance...
January 2017: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28544787/analysis-of-adults-with-congenital-heart-disease-presenting-to-pediatric-emergency-departments-with-arrhythmias
#5
Shaun Mohan, Brady S Moffett, Wilson Lam, Caridad de la Uz, Christina Miyake, Santiago O Valdes, Jeffrey J Kim
OBJECTIVE: As survivors of congenital heart disease (CHD) continue to age, healthcare utilization by this population has increased. It is unknown how often these patients utilize the emergency department (ED) at children's hospitals and how arrhythmias play a role in their utilization of care. DESIGN: Using a retrospective cohort design, the Pediatric Hospital Information System (PHIS) database was investigated and we studied adults (≥18 years) with CHD (ACHD) who presented to pediatric EDs from 2004 to 2014...
May 22, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28541122/chance-of-surgery-in-adult-congenital-heart-disease
#6
Carianne L Verheugt, Cuno Spm Uiterwaal, Ilonca Vaartjes, Enno T van der Velde, A C Zomer, Folkert J Meijboom, Petronella G Pieper, Marco C Post, Hubert W Vliegen, Mark G Hazekamp, Diederick E Grobbee, Barbara Jm Mulder
Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood. Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years...
January 1, 2017: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/28534239/analysis-of-screening-electrocardiogram-for-the-subcutaneous-defibrillator-in-adults-with-congenital-heart-disease
#7
Vincent C Thomas, Mark Peterson, Martin McDaniel, Humberto Restrepo, Abraham Rothman, Amit Jain
Candidates for the subcutaneous implantable cardioverter-defibrillator (S-ICD) are screened using an electrocardiogram (S-ECG) tool to measure appropriate detection. We sought to define the S-ICD candidacy of congenital heart disease patients using the S-ECG tool. We also analyzed the reliability of the (S-ECG) tool between measurers in this population. Patients above the age of 12 and with a diagnosis associated with either a higher incidence of cardiac arrest or vascular access challenges were asked to undergo screening...
May 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28527575/coup-tf-genes-human-diseases-and-the-development-of-the-central-nervous-system-in-murine-models
#8
Xiong Yang, Su Feng, Ke Tang
COUP-TFI and -TFII are members of the steroid/thyroid nuclear receptor superfamily. Recent clinical studies reveal that COUP-TFI gene mutations are associated with Bosch-Boonstra-Schaaf optic atrophy syndrome displaying symptoms of optic atrophy, intellectual disability, hypotonia, seizure, autism spectrum disorders, oromotor dysfunction, thin corpus callosum, or hearing defects, and COUP-TFII gene mutations lead to congenital heart defects and/or congenital diaphragmatic hernia with developmental delay and mental defects...
2017: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/28523852/preparing-adolescents-with-heart-problems-for-transition-to-adult-care-2009-2010-national-survey-of-children-with-special-health-care-needs
#9
Karrie F Downing, Matthew E Oster, Sherry L Farr
OBJECTIVE: A substantial percentage of children with congenital heart disease (CHD) fail to transfer to adult care, resulting in increased risk of morbidity and mortality. Transition planning discussions with a provider may increase rates of transfer, yet little is known about frequency and content of these discussions. We assessed prevalence and predictors of transition-related discussions between providers and parents of children with special healthcare needs (CSHCN) and heart problems, including CHD...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28523142/quality-of-life-an-underutilized-patient-reported-outcome-for-adults-with-congenital-heart-disease
#10
EDITORIAL
Sameh M Said, Joseph A Dearani
No abstract text is available yet for this article.
April 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28521893/pulmonary-hypertension-in-infants-children-and-young-adults
#11
REVIEW
Georg Hansmann
Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure...
May 23, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28520541/outcomes-and-prognostic-factors-for-adult-patients-with-congenital-heart-disease-undergoing-primary-or-reoperative-systemic-atrioventricular-valve-surgery
#12
Elizabeth H Stephens, Jiho Han, Jonathan Ginns, Marlon Rosenbaum, Paul Chai, Emile Bacha, David Kalfa
BACKGROUND: Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery. METHODS: We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation...
May 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28517030/gerbode-defect-and-multivalvular-dysfunction-complex-complications-in-adult-congenital-heart-disease
#13
Catarina Ruivo, Joana Guardado, Fernando Montenegro Sá, Fátima Saraiva, Alexandre Antunes, Joana Correia, João Morais
We report a clinical case of a 40-year-old male with surgically corrected congenital heart disease (CHD) 10 years earlier: closure of ostium primum, mitral annuloplasty, and aortic valve and root surgery. The patient was admitted with acute heart failure. Transesophageal echocardiography (TEE) revealed a dysmorphic and severely incompetent aortic valve, a partial tear of the mitral valve cleft repair and annuloplasty ring dehiscence. A true left ventricular-to-right atrial shunt confirmed a direct Gerbode defect...
May 18, 2017: Echocardiography
https://www.readbyqxmd.com/read/28508990/adolescents-with-congenital-heart-disease-their-opinions-about-the-preparation-for-transfer-to-adult-care
#14
Åsa Burström, Ewa-Lena Bratt, Björn Frenckner, Margret Nisell, Katarina Hanséus, Annika Rydberg, Maria Öjmyr-Joelsson
The aim of the study was to explore what adolescents with congenital heart disease (CHD) view as important in the preparation for the transfer to adult care. We performed interviews in four focus groups with adolescents (14-18 years old) at four university hospitals in Sweden. Data was analysed using qualitative content analysis. The analysis revealed one main category; Becoming a manager of the condition and four subcategories; Sufficient knowledge about the health, Be a participant in the care, Parental support, and Communicate with others about the health...
May 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28507990/development-of-an-online-evidence-based-patient-information-portal-for-congenital-heart-disease-a-pilot-study
#15
Jonathan R G Etnel, Arie P J van Dijk, Jolanda Kluin, Robin A Bertels, Elisabeth M W J Utens, Eugene van Galen, Ad J J C Bogers, Johanna J M Takkenberg
OBJECTIVES: In response to an increased need for patient information on congenital heart disease in the Netherlands, we initiated a nationwide initiative to develop an online, evidence-based patient information portal, starting with a pilot project aimed at the subgroup of patients with congenital aortic and pulmonary valve disease. METHODS AND RESULTS: We developed an information portal that aims to (1) improve patient knowledge and involvement and to subsequently reduce anxiety and decisional conflict and improve mental quality of life and (2) to support physicians in informing and communicating with their patients...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28506578/-congenital-heart-disease-in-adolescents-and-adults-management-in-a-general-cardiology-department-in-senegal
#16
A Mbaye, M Bodian, A A Ngaïdé, H Abdourafiq, M C B O Leye, S Savodogo, F Aw, M Ndiaye, I Kouamé, K Babaka, M Dioum, N D Gaye, S A Sarr, M B Ndiaye, A D Kane, A Kane
BACKGROUND: Congenital heart diseases in adults include malformations treated in childhood that decompensate secondarily and those asymptomatic at birth, appear later. This study aims to identify congenital heart diseases in adults in general cardiology department of Senegal and to assess clinical presentations, treatment and outcomes. METHODS: We conducted a cross-sectional and descriptive study based on the records of patients aged at least 16 years and followed for congenital heart disease in the cardiology department of the General Hospital of Grand-Yoff in Dakar between May 2003 and March 2015...
May 12, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28503552/impact-of-isolated-tricuspid-valve-repair-on-right-ventricular-remodelling-in-an-adult-congenital-heart-disease-population
#17
Roberto Marsico, Vito Domenico Bruno, Pierpaolo Chivasso, Anna Baritussio, Filippo Rapetto, Gustavo A Guida, Umberto Benedetto, Massimo Caputo
BACKGROUND: Surgical repair of isolated congenital tricuspid valve (TV) disease is rare with no well-defined indication and outcomes. Moreover, the role of right ventricle (RV) in this context has not yet been investigated. OBJECTIVES: We sought to assess the impact of congenital TV repair on cardiac remodelling and clinical-functional status and the importance of the RV function in an adult congenital heart disease (ACHD) population. METHODS AND RESULTS: From January 2005 to December 2015, 304 patients underwent TV surgery in our centre...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28501797/predictors-of-procedural-complications-in-adult-fontan-patients-undergoing-non-cardiac-procedures
#18
Alexander C Egbe, Arooj R Khan, Naser M Ammash, David W Barbara, William C Oliver, Sameh M Said, Emmanuel Akintoye, Carole A Warnes, Heidi M Connolly
OBJECTIVE: Limited data exist regarding the outcomes of non-cardiac procedures (NCPs) in adult patients after Fontan operations (Fontan patients). METHODS: To compare procedural outcomes after NCPs in Fontan patients with outcomes for two matched control groups: patients with repaired congenital heart disease and biventricular circulation (CHD-BiV) and patients with no heart disease (NHD). We defined cyanosis as oxygen saturation <90% and procedural hypoxia as saturation <80% or a decrease in saturation >10% from baseline...
May 13, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28497583/evaluation-of-athletes-with-complex-congenital-heart-disease
#19
Benjamin A Bates, Camille Richards, Michael Hall, Edmund K Kerut, William Campbell, Michael R McMullan
As a result of improvements in congenital heart surgery, there are more adults alive today with congenital heart disease (CHD) than children. Individuals with cardiac birth defects may be able to participate in physical activities but require proper cardiovascular evaluation. The American Heart Association and American College of Cardiology released guidelines in 2015 for athletes with cardiovascular abnormalities. The guidelines express that although restriction from competitive athletics may be indicated for some, the majority of individuals with CHD can and should engage in some form of physical activity...
May 12, 2017: Echocardiography
https://www.readbyqxmd.com/read/28496511/evaluation-of-coronary-arteries-in-non-ischemic-cardiomyopathies-a-case-report
#20
Farveh Vakilian, Mahmood Mohamadzadeh Shabestari, Ahmad Amin, Soheila Chamanian, Toktam Moghiman
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a congenital cardiac disease with myocardial involvement, most probably right ventricular (RV) dysfunction, accounting for 20% of sudden cardiac deaths. Characterized by the fibro-fatty infiltration of the RV free wall, ARVD/C presents in adolescents with ventricular arrhythmias and heart failure symptoms and as biventricular failure in adults. The coronary risk in these patients is not clear. We present an incidental finding: the left anterior descending artery cut-off in a middle-aged man with ARVD/C...
October 3, 2016: Journal of Tehran Heart Center
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