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https://www.readbyqxmd.com/read/28291606/geometry-and-growth-of-the-reconstructed-aorta-in-patients-with-hypoplastic-left-heart-syndrome-and-variants
#1
Christoph Haller, Devin Chetan, Arezou Saedi, Rachel Parker, Glen S Van Arsdell, Osami Honjo
OBJECTIVE: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. METHODS: A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre-stage II and pre-Fontan angiograms were measured...
February 14, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28275592/model-of-human-fetal-growth-in-hypoplastic-left-heart-syndrome-reduced-ventricular-growth-due-to-decreased-ventricular-filling-and-altered-shape
#2
Sukriti Dewan, Adarsh Krishnamurthy, Devleena Kole, Giulia Conca, Roy Kerckhoffs, Michael D Puchalski, Jeffrey H Omens, Heather Sun, Vishal Nigam, Andrew D McCulloch
INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a congenital condition with an underdeveloped left ventricle (LV) that provides inadequate systemic blood flow postnatally. The development of HLHS is postulated to be due to altered biomechanical stimuli during gestation. Predicting LV size at birth using mid-gestation fetal echocardiography is a clinical challenge critical to prognostic counseling. HYPOTHESIS: We hypothesized that decreased ventricular filling in utero due to mitral stenosis may reduce LV growth in the fetal heart via mechanical growth signaling...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28274521/cavopulmonary-anastomosis-during-same-hospitalization-as-stage-1-norwood-hybrid-palliative-surgery
#3
Sachin D Tadphale, Xinyu Tang, Nahed O ElHassan, Brandon Beam, Parthak Prodhan
BACKGROUND: Limited literature has examined characteristics of infants with hypoplastic left heart syndrome (HLHS) who remain hospitalized during the interstage period. We described their epidemiologic characteristics, in-hospital outcomes, and identified risk factors that predict the need for superior cavopulmonary anastomosis (SCPA) during the same hospitalization. METHODS: This retrospective multicenter database analysis included infants with HLHS who underwent stage 1 palliation from 2004 through 2013...
March 5, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28254208/molecular-cytogenetic-characterization-of-jacobsen-syndrome-11q23-3-q25-deletion-in-a-fetus-associated-with-double-outlet-right-ventricle-hypoplastic-left-heart-syndrome-and-ductus-venosus-agenesis-on-prenatal-ultrasound
#4
Chih-Ping Chen, Liang-Kai Wang, Pei-Chen Wu, Tung-Yao Chang, Schu-Rern Chern, Peih-Shan Wu, Yen-Ni Chen, Shin-Wen Chen, Chen-Chi Lee, Chien-Wen Yang, Wayseen Wang
OBJECTIVE: We present molecular cytogenetic characterization of Jacobsen syndrome (11q23.3-q25 deletion) in a fetus associated with double outlet right ventricle (DORV), hypoplastic left heart syndrome (HLHS), and ductus venosus (DV) agenesis on prenatal ultrasound. CASE REPORT: A 26-year-old woman underwent prenatal ultrasound examination at 22 weeks of gestation, which revealed intrauterine growth restriction, short femurs, DORV, HLHS, DV agenesis, single umbilical artery, and curly fourth toe of the left foot...
February 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28191731/right-ventricular-systolic-function-in-hypoplastic-left-heart-syndrome-a-comparison-of-manual-and-automated-software-to-measure-fractional-area-change
#5
Hanna K Ruotsalainen, Hannah R Bellsham-Revell, Aaron J Bell, Jaana I Pihkala, Tiina H Ojala, John M Simpson
BACKGROUND: Quantitative echocardiographic assessment of right ventricular function is important in children with hypoplastic left heart syndrome (HLHS). The aim of this study was to examine the repeatability of different echocardiographic techniques, both manual and automated, to measure fractional area change (FAC) in patients with HLHS and to correlate these measurements with magnetic resonance imaging (MRI)-derived ejection fraction (EF). METHODS: Fifty-one children with HLHS underwent transthoracic echocardiography and cardiac MRI under the same general anesthetic as part of routine inter-stage assessment...
February 13, 2017: Echocardiography
https://www.readbyqxmd.com/read/28142228/notch1-dependent-nitric-oxide-signaling-deficiency-in-hypoplastic-left-heart-syndrome-revealed-through-patient-specific-phenotypes-detected-in-bioengineered-cardiogenesis
#6
Sybil C L Hrstka, Xing Li, Timothy J Nelson
Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect (CHD) attributable to multifactorial molecular underpinnings. Multiple genetic loci have been implicated to increase the risk of disease, yet genotype-phenotype relationships remain poorly defined. Whole genome sequencing complemented by cardiac phenotype from five individuals in an HLHS-affected family enabled the identification of NOTCH1 as a prioritized candidate gene linked to CHD in three individuals with mutant allele burden significantly impairing Notch signaling in the HLHS-affected proband...
January 31, 2017: Stem Cells
https://www.readbyqxmd.com/read/28121380/practice-trends-over-time-in-the-care-of-infants-with-hypoplastic-left-heart-syndrome-a-report-from-the-national-pediatric-cardiology-quality-improvement-collaborative
#7
Waldemar F Carlo, James F Cnota, Robert J Dabal, Jeffrey B Anderson
OBJECTIVE: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras. DESIGN: Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008-1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014-11/2014) from these same centers...
January 25, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28118786/mathematical-analysis-and-physical-profile-of-blalock-taussig-shunt-and-sano-modification-procedure-in-hypoplastic-left-heart-syndrome
#8
Efrain Riveros Perez, Ricardo Riveros
The first stage of surgical treatment for hypoplastic left heart syndrome (HLHS) includes the creation of artificial systemic-to-pulmonary connections to provide pulmonary blood flow. The modified Blalock-Taussig (mBT) shunt has been the technique of choice for this procedure; however, a right ventricle-pulmonary artery (RV-PA) shunt has been introduced into clinical practice with encouraging but still conflicting outcomes when compared with the mBT shunt. The aim of this study is to explore mathematical modeling as a tool for describing physical profiles that could assist the surgical team in predicting complications related to stenosis and malfunction of grafts in an attempt to find correlations with clinical outcomes from clinical studies that compared both surgical techniques and to assist the anesthesiologist in making decisions to manage patients with this complex cardiac anatomy...
January 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
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
#9
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
#10
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...
March 1, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28024650/recoarctation-after-norwood-i-procedure-for-hypoplastic-left-heart-syndrome-impact-of-patch-material
#11
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...
February 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28018895/regional-differences-in-end-diastolic-volumes-between-3d-echo-and-cmr-in-hlhs-patients
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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...
February 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/27865477/tracheostomy-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-cardiac-operations-a-multicenter-analysis
#18
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
#19
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...
February 2017: 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
#20
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
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