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https://www.readbyqxmd.com/read/28100120/hybrid-stage-i-procedure-as-initial-palliation-for-neonate-with-hypoplastic-left-heart-syndrome-and-right-congenital-diaphragmatic-hernia
#1
Draginja Cvetkovic, Joseph Giamelli, Michael Lyew, Markus Erb, Suvro Sett, Youmna DiStefano
During the past decade, a hybrid procedure has emerged and dramatically evolved as an alternative stage I palliation to the conventional Norwood procedure in neonates with hypoplastic left heart syndrome (HLHS). The hybrid approach avoids the need for cardiopulmonary bypass (CPB) utilizing stenting of the arterial duct and bilateral pulmonary artery banding. Cerebral and coronary perfusion pressure is maintained, and the pulmonary vasculature is protected from higher systemic pressure. Elimination of risks associated with CPB gains vital time to stabilize the patient and correct coexisting noncardiac anomalies and allows growth in preparation for the later stages of the Fontan pathway...
January 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28043677/pressure-volume-loop-derived-cardiac-indices-during-dobutamine-stress-a-step-towards-understanding-limitations-in-cardiac-output-in-children-with-hypoplastic-left-heart-syndrome
#2
James Wong, Kuberan Pushparajah, Adelaide de Vecchi, Bram Ruijsink, Gerald F Greil, Tarique Hussain, Reza Razavi
BACKGROUND: Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies. METHODS: Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6years (range 3.5-11.6years), mean time since Fontan completion 5...
December 22, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28024650/recoarctation-after-norwood-i-procedure-for-hypoplastic-left-heart-syndrome-impact-of-patch-material
#3
Keti Vitanova, Julie Cleuziou, Jelena Pabst von Ohain, Melchior Burri, Andreas Eicken, Rüdiger Lange
BACKGROUND: The development of recoarctation after the Norwood I procedure is a known complication in patients with hypoplastic left heart syndrome (HLHS). METHODS: We reviewed data on 194 consecutive patients with HLHS who underwent the Norwood I procedure between 2000 and 2015. All patients who survived until the second stage of the procedure were included. Recoarctation was defined by the need for intervention, either catheter based or surgical. The aim of our study was to determine if the patch material used for the initial arch reconstruction had an influence on the development of recoarctation...
December 23, 2016: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28018895/regional-differences-in-end-diastolic-volumes-between-3d-echo-and-cmr-in-hlhs-patients
#4
Alberto Gomez, Ozan Oktay, Daniel Rueckert, Graeme P Penney, Julia A Schnabel, John M Simpson, Kuberan Pushparajah
Ultrasound is commonly thought to underestimate ventricular volumes compared to magnetic resonance imaging (MRI), although the reason for this and the spatial distribution of the volume difference is not well understood. In this paper, we use landmark-based image registration to spatially align MRI and ultrasound images from patients with hypoplastic left heart syndrome and carry out a qualitative and quantitative spatial comparison of manual segmentations of the ventricular volume obtained from the respective modalities...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28007262/off-pump-atrial-septectomy-for-infants-with-restrictive-atrial-septal-defect
#5
Tai Fuchigami, Masahiko Nishioka, Toru Akashige, Shotaro Higa, Nobuhiro Nagata
The management of some subsets of patients with an unsuccessful balloon atrial septostomy (BAS) and poor general condition is typically challenging. Our novel technique involves off-pump atrial septectomy using laparoscopic forceps guided by intraoperative direct pericardial echocardiography. The technique was first used in a case of tricuspid atresia in a patient in preshock and was then used in 2 more patients with hypoplastic left heart syndrome (HLHS) (age, 10-116 days; body weight, 2.4-3.7 kg). There were no operative complications of embolism or bleeding in our series...
January 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/27998845/initial-experience-with-live-three-dimensional-image-overlay-for-ductal-stenting-in-hypoplastic-left-heart-syndrome
#6
Sebastian Goreczny, Gareth J Morgan, Pawel Dryzek, Jadwiga A Moll, Tomasz Moszura
AIMS: Precise visualisation of ductal morphology and adjacent vessels is crucial for accurate stent choice and placement during hybrid palliation of hypoplastic left heart syndrome (HLHS). We aimed to describe our initial experience with live three-dimensional reconstruction overlay derived from rotational angiography (RA) for ductal stenting in HLHS. METHODS AND RESULTS: We carried out a retrospective review of ductal stenting in 18 newborns with HLHS, including six patients with 3D reconstruction overlay used to guide the intervention...
December 20, 2016: EuroIntervention
https://www.readbyqxmd.com/read/27958617/results-of-the-fontan-operation-with-no-early-mortality-in-248-consecutive-patients
#7
Edward Malec, Christoph Schmidt, Anja Lehner, Katarzyna Januszewska
BACKGROUND: Fontan operation has undergone several modifications and today is the primary way to treat a broad spectrum of congenital heart defects AIM: The purpose of this study is to present the results of treatment of children with a single ventricle operated by the same surgical team and managed according to a uniform strategy. METHODS: In the years 2007-2015, in 248 children aged 3.7 ± 2.6 years and weigh of 14.6 ± 6.1 kg with a single ventricle the Fontan surgery was performed...
December 13, 2016: Kardiologia Polska
https://www.readbyqxmd.com/read/27894567/proportion-of-selected-congenital-heart-defects-attributable-to-recognized-risk-factors
#8
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
#9
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
#10
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
#11
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
#12
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...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27803957/interstage-survival-for-patients-with-hypoplastic-left-heart-syndrome-after-ecmo
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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...
December 2016: Echocardiography
https://www.readbyqxmd.com/read/27592606/looks-do-matter-aortic-arch-shape-after-hypoplastic-left-heart-syndrome-palliation-correlates-with-cavopulmonary-outcomes
#19
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
#20
REVIEW
Jessica M Reed
No abstract text is available yet for this article.
September 2016: World Journal for Pediatric & Congenital Heart Surgery
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