Read by QxMD icon Read


Christopher E Greenleaf, J Miguel Urencio, Jorge D Salazar, Ali Dodge-Khatami
Since the first successful intervention for hypoplastic left heart syndrome (HLHS) was undertaken by Norwood in 1983, there have been many advancements in the pre-, intra-, and postoperative care of these children for a diagnosis that just 25 years ago was almost certainly a fatal one. This paper aims to describe the most recent trends and perspectives on the treatment of HLHS. In particular, we will discuss the five current options for HLHS, including Norwood stage I as the beginning to 3-stage palliation, transplant, true hybrid, hybrid-bridge-to-Norwood, and compassionate care...
July 2016: Translational pediatrics
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
David J Goldberg, Benjamin French, Anita L Szwast, Michael G McBride, Stephen M Paridon, Jack Rychik, Laura Mercer-Rosa
OBJECTIVE: Tricuspid annular plane systolic excursion (TAPSE) is a reproducible measure of right ventricular systolic function. We sought to determine the reliability of TAPSE measurements, to evaluate TAPSE in patients with hypoplastic left heart syndrome (HLHS) relative to normal values, and to correlate values of TAPSE with measures of exercise performance. DESIGN: Tricuspid annular plane systolic excursion measurements were performed off-line in the cohort of patients with single right ventricle anatomy who had been enrolled in the Sildenafil After Fontan Operation clinical trial...
September 5, 2016: Echocardiography
Jan L Bruse, Elena Cervi, Kristin McLeod, Giovanni Biglino, Maxime Sermesant, Xavier Pennec, Andrew M Taylor, Silvia Schievano, Tain-Yen Hsia
BACKGROUND: Aortic arch reconstruction after hypoplastic left heart syndrome (HLHS) palliation can vary widely in shape and dimensions between patients. Arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch three-dimensional shape features with functional and short-term outcome data after total cavopulmonary connection (TCPC). METHODS: Aortic arch shape models of 37 patients with HLHS (age, 2.89 ± 0.99 years) were reconstructed from magnetic resonance data before TCPC completion...
September 1, 2016: Annals of Thoracic Surgery
Jessica M Reed
No abstract text is available yet for this article.
September 2016: World Journal for Pediatric & Congenital Heart Surgery
Meaghan M Mackesy, Brian T Kalish, Wayne Tworetzky, Stephen Sanders, Alyaa Al-Ibraheemi, Louise Wilkins-Haug, James Lock, Audrey Marshall, Carol B Benson
Fetuses with hypoplastic left heart syndrome (HLHS) and intact atrial septum are a particular subset of HLHS neonates with high perinatal mortality. The reported mortality in these patients is 50% to 70%, even with prenatal diagnosis. Prenatal left atrial and pulmonary venous hypertension results in abnormal pulmonary vascular and parenchymal development. The goal of this study was to compare the sonographic appearance of the lungs in fetuses with HLHS/intact atrial septum to neonatal outcome and/or pathology in cases where in utero intervention was performed to open the atrial septum...
August 29, 2016: Ultrasound Quarterly
Erin A Paul, Kristina Orfali, Thomas J Starc
We hypothesized that enthusiasm for surgery increased for infants with hypoplastic left heart syndrome (HLHS) at Columbia University Medical Center (CUMC) between 1995 and 2012. We sought to identify factors that engendered this paradigm shift. Confidential surveys were distributed to providers at CUMC in 1995 and 2012 to measure enthusiasm for surgical intervention for HLHS. Surgical preference scores are presented as median [interquartile range]. Surveys were completed by 99/176 providers (56 % response rate) in 1995 and 153/267 (57 %) in 2012...
August 27, 2016: Pediatric Cardiology
Sunil K Verma, Vaibhav Deshmukh, Curtis A Nutter, Elizabeth Jaworski, Wenhao Jin, Lalita Wadhwa, Joshua Abata, Marco Ricci, Joy Lincoln, James F Martin, Gene W Yeo, Muge N Kuyumcu-Martinez
Hypoplastic left heart syndrome (HLHS) is a fatal congenital heart disease in which the left side of the heart is underdeveloped, impairing the systemic circulation. Underdeveloped left ventricle exerts biomechanical stress on the right ventricle that can progress into heart failure. Genome-wide transcriptome changes have been identified at early stages in the right ventricle (RV) of infants with HLHS, although the molecular mechanisms remain unknown. Here, we demonstrate that the RNA binding protein Rbfox2, which is mutated in HLHS patients, is a contributor to transcriptome changes in HLHS patient RVs...
2016: Scientific Reports
Nikita M Patel, Mohamed A Mohamed, Iman K Yazdi, Ennio Tasciotti, Ravi K Birla
Current treatments in hypoplastic left heart syndrome (HLHS) include multiple surgeries to refunctionalize the right ventricle and/or transplant. The development of a tissue-engineered left ventricle (LV) would provide a therapeutic option to overcome the inefficiencies and limitations associated with current treatment options. This study provides a foundation for the development and fabrication of the bioengineered open ventricle (BEOV) model. BEOV molds were developed to emulate the human LV geometry; molds were used to produce chitosan scaffolds...
July 20, 2016: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
Jeffrey Moore, Diane Paulus, Clifford L Cua, Naomi J Kertesz, Yongjie Miao, John P Cheatham, Mark Galantowicz, Richard Fernandez
The hybrid procedure is an alternative palliative strategy for patients with single-ventricle physiology. No data exist documenting the incidence of arrhythmias after the hybrid procedure. Goal of this study was to determine the incidence and type of arrhythmias in patients undergoing the hybrid procedure. A retrospective chart review was performed including all patients undergoing the hybrid procedure between January of 2010 through December of 2013. Sixty-five patients underwent the hybrid procedure during this time period (43 HLHS, 22 other)...
July 18, 2016: Pediatric Cardiology
James Wong, Pablo Lamata, Rahul H Rathod, Sophie Bertaud, Nathalie Dedieu, Hannah Bellsham-Revell, Kuberan Pushparajah, Reza Razavi, Tarique Hussain, Tobias Schaeffter, Andrew J Powell, Tal Geva, Gerald F Greil
OBJECTIVES: The Norwood procedure for hypoplastic left heart syndrome (HLHS) is performed either via a right ventricle-to-pulmonary artery (RVPA) conduit or a modified Blalock-Taussig (MBT) shunt. Cardiac magnetic resonance (CMR) data was used to assess the effects of the RVPA conduit on ventricular shape and function through a computational analysis of anatomy and assessment of indices of strain. METHODS: A retrospective analysis of 93 CMR scans of subjects with HLHS was performed (59 with MBT shunt, 34 with RVPA conduit), incorporating data at varying stages of surgery from two congenital centres...
July 15, 2016: European Journal of Cardio-thoracic Surgery
Varsha Thakur, Nadia Munk, Luc Mertens, Lynne E Nield
OBJECTIVE: Hypoplastic left heart syndrome is frequently diagnosed prenatally with variable benefit. We performed a systematic review to evaluate the impact of fetal diagnosis; the primary objective was to evaluate impact on mortality. METHODS: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed. Seven databases were searched. Meta-analysis was performed using a random effects model to evaluate the effect of fetal diagnosis on mortality...
September 2016: Prenatal Diagnosis
Danièle Klett, Philippine Meslin, Lauriane Relav, Thi Mong Diep Nguyen, Julie Mariot, Gwenhaël Jégot, Claire Cahoreau, Yves Combarnous
In order to study the intracellular cAMP response kinetics of Leydig cells to hormones with LH activity, we used MLTC-1 cells transiently expressing a chimeric cAMP-responsive luciferase so that real-time variations of intracellular cAMP concentration could be followed using oxiluciferin luminescence produced from catalyzed luciferin oxidation. The potencies of the different LHs and CGs were evaluated using areas under the curves (AUC) of their kinetics over 60 min stimulation. All mammalian LHs and CGs tested were found to stimulate cAMP accumulation in these cells...
October 15, 2016: Molecular and Cellular Endocrinology
Douglas Y Mah, Henry Cheng, Mark E Alexander, Lynn Sleeper, Jane W Newburger, Pedro J Del Nido, Ravi R Thiagarajan, Satish K Rajagopal
OBJECTIVES: Publicly available data from the Pediatric Heart Network's Single Ventricle Reconstruction Trial was analyzed to determine the prevalence, timing, risk factors for, and impact of second- and third-degree heart block (HB) on outcomes in patients who underwent stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS). METHODS: The presence and date of onset of post-S1P HB occurring within the first year of life, potential risk factors for HB, and factors known to predict poor outcomes after S1P were extracted...
July 2016: Journal of Thoracic and Cardiovascular Surgery
Elizabeth C Caris, Nicole Dempster, Gil Wernovsky, Catherine Butz, Trent Neely, Robin Allen, Jamie Stewart, Holly Miller-Tate, Rachel Fonseca, Karen Texter, Lisa Nicholson, Clifford L Cua
OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed...
June 20, 2016: Congenital Heart Disease
Miriam Michel, Jana Logoteta, Andreas Entenmann, Jan Hinnerk Hansen, Inga Voges, Hans-Heiner Kramer, Colin Petko
Accurate assessment of ventricular function is particularly important in children with hypoplastic left heart syndrome (HLHS) after completion of the total cavopulmonary connection (TCPC). For this purpose, two-dimensional speckle tracking (2DST) is a promising technique as it does not depend on the angle of insonation or the geometry of the ventricle. The objective of this study was to assess changes in systolic and diastolic right ventricular (RV) function within a 5-year follow-up period of HLHS patients after TCPC using conventional and 2DST echocardiography...
October 2016: Pediatric Cardiology
Josue Chery, Joshua Wong, Shan Huang, Shuyun Wang, Ming-Sing Si
Hypoplastic left heart syndrome (HLHS), the most severe and common form of single ventricle congenital heart lesions, is characterized by hypoplasia of the mitral valve, left ventricle (LV), and all LV outflow structures. While advances in surgical technique and medical management have allowed survival into adulthood, HLHS patients have severe morbidities, decreased quality of life, and a shortened lifespan. The single right ventricle (RV) is especially prone to early failure because of its vulnerability to chronic pressure overload, a mode of failure distinct from ischemic cardiomyopathy encountered in acquired heart disease...
June 28, 2016: Tissue Engineering. Part B, Reviews
Laura K Diaz, J William Gaynor, Shannon J Koh, Richard F Ittenbach, Marsha Gerdes, Judy C Bernbaum, Elaine H Zackai, Robert R Clancy, Mohamed A Rehman, Jeffrey W Pennington, Nancy Burnham, Thomas L Spray, Susan C Nicolson
OBJECTIVES: Despite improved survival in children with hypoplastic left heart syndrome (HLHS), significant concern persists regarding their neurodevelopmental (ND) outcomes. Previous studies have identified patient factors, such as prematurity and genetic syndromes, to be associated with worse ND outcomes. However, no consistent relationships have been identified among modifiable management factors, including cardiopulmonary bypass strategies, and ND outcomes after cardiac surgery in infancy...
August 2016: Journal of Thoracic and Cardiovascular Surgery
David A Danford, Quentin Karels, Shelby Kutty
OBJECTIVE: Our objective was to characterise the divergence of effort from outcome in congenital heart disease (CHD) care by measuring mortality-related resource utilisation fraction (MRRUF) for various CHD lesions across institutions of differing volumes. METHODS: Study design was observational analysis of an administrative database, the Pediatric Health Information System (PHIS). The setting was inpatient; 2004-2013. Patients were ≤21 years old with atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF), hypoplastic left heart syndrome (HLHS) or other single ventricle (SV)...
2016: Open Heart
Haitham Torky, Asem Anwar, Alaa Elebrashy, Fahd Elomda
OBJECTIVES: To determine the prenatal incidence of moderate and severe cardiac anomalies and determine the prenatal pattern of CHD. STUDY DESIGN: Multi-center retrospective observational study. SETTINGS: Cairo, Al-Azhar and 6th October Universities. PATIENTS AND METHODS: 5499 pregnant women in the second and third trimester attending the fetal medicine unit in the three universities from January 2012 to June 2013 for anomaly scan were retrospectively reviewed...
April 2016: Pregnancy Hypertension
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"