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https://www.readbyqxmd.com/read/27894567/proportion-of-selected-congenital-heart-defects-attributable-to-recognized-risk-factors
#1
Regina M Simeone, Sarah C Tinker, Suzanne M Gilboa, A J Agopian, Matthew E Oster, Owen J Devine, Margaret A Honein
PURPOSE: To assess the contribution of multiple risk factors for two congenital heart defects-hypoplastic left heart syndrome (HLHS) and tetralogy of Fallot (TOF). METHODS: We used data from the National Birth Defects Prevention Study (1997-2011) to estimate average adjusted population attributable fractions for several recognized risk factors, including maternal prepregnancy overweight-obesity, pregestational diabetes, age, and infant sex. RESULTS: There were 594 cases of isolated simple HLHS, 971 cases of isolated simple TOF, and 11,829 controls in the analysis...
December 2016: Annals of Epidemiology
https://www.readbyqxmd.com/read/27885446/post-cardiotomy-rescue-extracorporeal-cardiopulmonary-resuscitation-in-neonates-with-single-ventricle-after-intractable-cardiac-arrest-attrition-after-hospital-discharge-and-predictors-of-outcome
#2
Anastasios C Polimenakos, Vincent Rizzo, Chawki F El-Zein, Michel N Ilbawi
Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates...
November 24, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27865477/tracheostomy-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-cardiac-operations-a-multicenter-analysis
#3
Parthak Prodhan, Amit Agarwal, Nahed O ElHassan, Elijah H Bolin, Brandon Beam, Xiomara Garcia, Michael Gaies, Xinyu Tang
BACKGROUND: Less than 2.7% of infants undergoing congenital heart disease operations have difficulty weaning from invasive mechanical ventilation. In such instances, clinicians may choose to perform tracheostomy. Limited literature has examined tracheostomy placement specifically in infants with hypoplastic left heart syndrome (HLHS). This study evaluated the risk factors for tracheostomy placement in infants with HLHS and examined the outcomes of these infants before their first hospital discharge...
November 16, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27817953/mechanically-assisted-bidirectional-cavopulmonary-shunt-in-neonates-and-infants-an-acute-human-pilot-study
#4
Osami Honjo, Sandra L Merklinger, John B Poe, Anne-Marie Guerguerian, Hargen Zhang, Katherine L Taylor, Glen S Van Arsdell
OBJECTIVE: Poor survival following surgical palliation for hypoplastic left heart syndrome (HLHS) raises the question of the need for a paradigm shift. This is the first human study to investigate the possibility of primary "in-series" palliation in neonates and infants with HLHS in an acute setting with the aid of 2 types of mechanical assist: superior vena cava (SVC)-to-pulmonary artery (PA) pump assist and SVC-to-right atrium (RA) oxygenation assist. METHODS: By rearranging the cannula sites and flow rates for modified ultrafiltration, 2 types of mechanically assisted bidirectional cavopulmonary shunt (BCPS) circulation were simulated for 20 minutes...
September 28, 2016: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27816303/ventricular-function-and-ventriculo-arterial-coupling-after-palliation-of-hypoplastic-left-heart-syndrome-a-comparative-study-with-fontan-patients-with-lv-morphology
#5
J Logoteta, C Ruppel, J H Hansen, G Fischer, K Becker, H-H Kramer, A Uebing
BACKGROUND: Conceptually the right ventricle (RV) is less suitable to support the Fontan circulation than the left (LV). After palliation of hypoplastic left heart syndrome (HLHS) involving aortic reconstruction during the Norwood procedure the RV is exposed to abnormal afterload. We studied ventricular function and ventriculo-arterial coupling in HLHS patients (RV) and Fontan patients with single LV morphology that did (LV+N) and did not (LV-N) undergo Norwood-type aortic reconstruction...
October 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27803957/interstage-survival-for-patients-with-hypoplastic-left-heart-syndrome-after-ecmo
#6
Richard P Fernandez, Brian F Joy, Robin Allen, Jamie Stewart, Holly Miller-Tate, Yongjie Miao, Lisa Nicholson, Clifford L Cua
There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival...
November 1, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27789736/impact-of-myh6-variants-in-hypoplastic-left-heart-syndrome
#7
Aoy Tomita-Mitchell, Karl D Stamm, Donna K Mahnke, Min-Su Kim, Pip M Hidestrand, Huan Ling Liang, Mary A Goetsch, Mats Hidestrand, Pippa Simpson, Andrew N Pelech, James S Tweddell, D Woodrow Benson, John W Lough, Michael E Mitchell
Hypoplastic left heart syndrome (HLHS) is a clinically and anatomically severe form of congenital heart disease (CHD). Although prior studies suggest that HLHS has a complex genetic inheritance, its etiology remains largely unknown. The goal of this study was to characterize a risk gene in HLHS and its effect on HLHS etiology and outcome. We performed next-generation sequencing on a multigenerational family with a high prevalence of CHD/HLHS, identifying a rare variant in the α-myosin heavy chain (MYH6) gene...
December 1, 2016: Physiological Genomics
https://www.readbyqxmd.com/read/27787502/ciliopathy-variant-burden-and-developmental-delay-in-children-with-hypoplastic-left-heart-syndrome
#8
Gabrielle C Geddes, Karl Stamm, Michael Mitchell, Kathleen A Mussatto, Aoy Tomita-Mitchell
PURPOSE: To test the hypothesis that patients with hypoplastic left heart syndrome (HLHS) and developmental delay will have a higher average summative C-score in ciliopathy genes than patients with HLHS without developmental delay. METHODS: Ciliopathy gene variant burden was determined utilizing a summative C-score for 14 ciliopathy genes in children with HLHS (n = 24). Mean summative C-scores were compared between children with and without developmental delay. Genome-wide randomizing gene sets were evaluated as a scoring control...
October 27, 2016: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/27709095/hypoplastic-left-heart-syndrome-current-perspectives
#9
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
https://www.readbyqxmd.com/read/27685952/a-population-based-analysis-of-mortality-in-patients-with-turner-syndrome-and-hypoplastic-left-heart-syndrome-using-the-texas-birth-defects-registry
#10
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
https://www.readbyqxmd.com/read/27596010/tricuspid-annular-plane-systolic-excursion-correlates-with-exercise-capacity-in-a-cohort-of-patients-with-hypoplastic-left-heart-syndrome-after-fontan-operation
#11
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
https://www.readbyqxmd.com/read/27592606/looks-do-matter-aortic-arch-shape-after-hypoplastic-left-heart-syndrome-palliation-correlates-with-cavopulmonary-outcomes
#12
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
https://www.readbyqxmd.com/read/27587506/perspectives-on-hlhs-from-a-pediatric-cardiology-nurse
#13
REVIEW
Jessica M Reed
No abstract text is available yet for this article.
September 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27575842/sonographic-pulmonary-abnormalities-in-fetuses-with-hypoplastic-left-heart-syndrome-and-intact-atrial-septum-undergoing-attempted-atrial-septostomy-in-utero
#14
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
https://www.readbyqxmd.com/read/27567909/hypoplastic-left-heart-syndrome-exploring-a-paradigm-shift-in-favor-of-surgery
#15
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...
December 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27485310/rbfox2-function-in-rna-metabolism-is-impaired-in-hypoplastic-left-heart-syndrome-patient-hearts
#16
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
https://www.readbyqxmd.com/read/27438342/the-design-and-fabrication-of-a-three-dimensional-bioengineered-open-ventricle
#17
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
https://www.readbyqxmd.com/read/27425423/arrhythmias-after-stage-i-hybrid-palliation-in-single-ventricle-patients
#18
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)...
December 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27422888/right-ventricular-morphology-and-function-following-stage-i-palliation-with-a-modified-blalock-taussig-shunt-versus-a-right-ventricle-to-pulmonary-artery-conduit
#19
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
https://www.readbyqxmd.com/read/27416335/does-prenatal-diagnosis-of-hypoplastic-left-heart-syndrome-make-a-difference-a-systematic-review
#20
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
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