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Congenital Heart Disease

Diego A Lara, Mary K Ethen, Mark A Canfield, Wendy N Nembhard, Shaine A Morris
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population. METHODS: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999-2007. We used Kaplan-Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS-)...
September 29, 2016: Congenital Heart Disease
Giovanni Biglino, Claudio Capelli, Despina Koniordou, Di Robertshaw, Lindsay-Kay Leaver, Silvia Schievano, Andrew M Taylor, Jo Wray
BACKGROUND: Nurse education and training are key to providing congenital heart disease (CHD) patients with consistent high standards of care as well as enabling career progression. One approach for improving educational experience is the use of 3D patient-specific models. OBJECTIVES: To gather pilot data to assess the feasibility of using 3D models of CHD during a training course for cardiac nurses; to evaluate the potential of 3D models in this context, from the nurses' perspective; and to identify possible improvements to optimise their use for teaching...
September 26, 2016: Congenital Heart Disease
Raya Safa, Ronald Thomas, Peter P Karpawich
BACKGROUND: The early ECG repolarization QRS pattern (ERp), with J-point elevation of 0.1 mV in two contiguous inferior and/or lateral leads, can be associated with ventricular arrhythmias among adults. The significance of an ERp in the young is unknown. OBJECTIVE: The purpose of this study was to assess the prevalence of ERp among young patients (pts), describe and correlate the characteristics with clinical presentations and any arrhythmias. METHODS: This was a 1 y retrospective review of ECGs obtained from patients referred specifically for documented arrhythmias, possible arrhythmia-related symptoms or sports clearance...
September 21, 2016: Congenital Heart Disease
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...
September 21, 2016: Congenital Heart Disease
Benjamin H Goot, Sonali Patel, Brian Fonseca
OBJECTIVE: When imaging the lower airway by MRI, the traditional technique turbo spin echo (TSE) results in high quality 2D images, however planning and acquisition times are lengthy. An alternative, delayed volume interpolated breath-holds examination (VIBE), is a 3D gradient echo technique that produces high spatial resolution imaging of the airway in one breath-hold. The objective of this study is to retrospectively evaluate the accuracy of lower airway measurements obtained by delayed VIBE when compared to TSE...
September 9, 2016: Congenital Heart Disease
Nicola Maschietto, Luca Semplicini, Giulio Ceolotto, Arianna Cattelan, Helen Poser Dvm, Ilaria Iacopetti, Gabriele Gerardi, Giulia Maria De Benedictis, Tommaso Pilla, Daniele Bernardini, Luca Aresu, Stefania Rizzo, Cristina Basso, Andrea Semplicini, Ornella Milanesi
BACKGROUND: Stent implantation is the treatment of choice for adolescents and adults with aortic coarctation (CoAo). Despite excellent short-term results, 20%-40% of the patients develop arterial hypertension later in life, which was attributed to inappropriate response of the aortic baroreceptors to increased stiffness of the ascending aorta (ASAO), either congenital or induced by CoAo repair. In particular, it has been hypothesized that stent itself may cause or sustain hypertension...
September 7, 2016: Congenital Heart Disease
Camden Hebson, Wendy Book, Robert W Elder, Ryan Ford, Maan Jokhadar, Kirk Kanter, Brian Kogon, Adrienne H Kovacs, Rebecca D Levit, Michael Lloyd, Kevin Maher, Preeti Reshamwala, Fred Rodriguez, Rene Romero, Thor Tejada, Anne Marie Valente, Gruschen Veldtman, Michael McConnell
"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions...
September 6, 2016: Congenital Heart Disease
Jonathan Komisar, Shubhika Srivastava, Miwa Geiger, John Doucette, Helen Ko, Jay Shenoy, Rajesh Shenoy
BACKGROUND: Antenatal diagnosis of congenital heart defects (CHD) can impact outcomes in neonates with severe CHD. Obstetric screening guidelines and the indications for fetal echocardiography (FE) have evolved in an attempt to improve the early prenatal detection of CHD. Analyzing yield for specific indications will help clinicians better stratify at-risk pregnancies. METHODS: Retrospective cohort study of all FE performed between 2000 and 2010 at a single tertiary care academic medical center in New York City...
August 26, 2016: Congenital Heart Disease
Lianne M Geerdink, Gideon J du Marchie Sarvaas, Irene M Kuipers, Willem A Helbing, Tammo Delhaas, Henriette Ter Heide, Lieke Rozendaal, Chris L de Korte, Sandeep K Singh, Tjark Ebels, Mark G Hazekamp, Felix Haas, Ad J J C Bogers, Livia Kapusta
OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study. DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed...
August 25, 2016: Congenital Heart Disease
Richard J Dobson, Ify Mordi, Mark H Danton, Niki L Walker, Hamish A Walker, Nikolaos Tzemos
OBJECTIVE: Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes. DESIGN: Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29...
August 24, 2016: Congenital Heart Disease
Kavitha N Pundi, Krishna N Pundi, Jonathan N Johnson, Joseph A Dearani, Zhuo Li, David J Driscoll, Philip L Wackel, Christopher J McLeod, Frank Cetta, Bryan C Cannon
OBJECTIVES: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation. BACKGROUND: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation. METHODS: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution...
August 22, 2016: Congenital Heart Disease
Christoph Gräni, Dominik C Benz, Mathias Possner, Olivier F Clerc, Fran Mikulicic, Jan Vontobel, Julia Stehli, Tobias A Fuchs, Aju P Pazhenkottil, Oliver Gaemperli, Philipp A Kaufmann, Ronny R Buechel
OBJECTIVE: To provide data on the value of fused cardiac hybrid imaging with coronary computed tomography angiography (CCTA) and positron emission tomography myocardial perfusion imaging (PET-MPI) in patients with complex coronary artery anomalies (CCAA). DESIGN/SETTING: This is a retrospective, single-center study. PATIENTS: Seven consecutive patients with CCAA (mean 57 ± 7 y, 86% were male) who underwent clinically indicated hybrid CCTA/PET-MPI between 2005 and 2015 in our clinic were included...
August 19, 2016: Congenital Heart Disease
Saar Danon, Robert G Gray, Matthew A Crystal, Gareth Morgan, Daniel H Gruenstein, Bryan H Goldstein, Brent M Gordon
OBJECTIVE: Intravascular stents are now routinely used to treat young patients with vascular stenoses. Future stent redilations are often necessary to account for somatic growth. The purpose of this study was to compile a database of characteristics for stents commonly used in the treatment of congenital heart disease patients, and compare serial dilation to direct dilation to the maximal diameter. DESIGN: A standardized bench testing protocol was established and utilized in the assessment of all stents...
July 27, 2016: Congenital Heart Disease
(no author information available yet)
No abstract text is available yet for this article.
September 2016: Congenital Heart Disease
Douglas S Moodie
No abstract text is available yet for this article.
September 2016: Congenital Heart Disease
Jaeger P Ackerman, John A Smestad, David J Tester, Muhammad Y Qureshi, Beau A Crabb, Nancy J Mendelsohn, Michael J Ackerman
OBJECTIVE: To use whole exome sequencing (WES) of a family trio to identify a genetic cause for polyvalvular syndrome. METHODS AND RESULTS: A male child was born with mild pulmonary valve stenosis and mild aortic root dilatation, and an atrial septal defect, ventricular septal defect, and patent ductus arteriosus that were closed surgically. Subsequently, the phenotype of polyvalvular syndrome with involvement of both semilunar and both atrioventricular valves emerged...
September 2016: Congenital Heart Disease
Robert P Richter, Mark A Law, Santiago Borasino, Jessica A Surd, Jeffrey A Alten
OBJECTIVE: To describe a novel real-time ultrasound (US)-guided distal superficial femoral vein (DSFV) cannulation technique for insertion of peripherally inserted central catheters (PICC) in critically ill infants with congenital heart disease. DESIGN: Descriptive retrospective cohort study SETTING: Pediatric cardiac intensive care unit in a pediatric tertiary hospital PATIENTS: First 28 critically ill infants that received DSFV PICCs via this new technique. RESULTS: Thirty-seven US-guided DSFV PICCs were attempted on 31 infants from September 2012 to November 2014; 34 PICCs were placed in 28 patients (success rate 92%)...
July 20, 2016: Congenital Heart Disease
Benjamin Acheampong, Jonathan N Johnson, John M Stulak, Joseph A Dearani, Sudhir S Kushwaha, Richard C Daly, Dawit T Haile, Gregory J Schears
BACKGROUND: Cardiac operations in high-risk adult congenital heart disease (ACHD) patients may require mechanical circulatory support (MCS), such as extracorporeal membrane oxygenation (ECMO) or intraaortic balloon pump (IABP), to allow the cardiopulmonary system to recover. METHODS: We reviewed records for all ACHD patients who required MCS following cardiotomy at our institution from 1/2001 to 12/2013. RESULTS: During the study period, 2264 (mean age 39...
July 20, 2016: Congenital Heart Disease
Hoi Lam She, Arno A W Roest, Emmeline E Calkoen, Pieter J van den Boogaard, Rob J van der Geest, Mark G Hazekamp, Albert de Roos, Jos J M Westenberg
PURPOSE: To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan's operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach. METHOD: Seven patients with functional univentricular heart after Fontan's operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured...
July 18, 2016: Congenital Heart Disease
Rohit S Loomba, Gabrielle C Geddes, Donald Basel, Dudley W Benson, Steven R Leuthner, David A Hehir, Nancy Ghanayem, Amanda J Shillingford
Heterotaxy (HTX) is a laterality defect resulting in abnormal arrangement of the thoracic and abdominal organs across the right-left axis, and is associated with multiple anatomic and physiologic disruptions. HTX often occurs in association with complex congenital heart disease. Splenic abnormalities are also common and convey an increased risk of bacteremia (bacteremia) with a high associated mortality. We performed a systematic review of the literature studying the risk of infection in HTX patients and strategies that can be utilized to prevent such infections...
July 18, 2016: Congenital Heart Disease
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