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Hypoplastic heart

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https://www.readbyqxmd.com/read/28217680/first-reported-case-of-fetal-aortic-valvuloplasty-in-asia
#1
Sun-Young Yoon, Hye-Sung Won, Mi-Young Lee, Min Kyong Cho, Euiseok Jung, Ki-Soo Kim, Young-Hwue Kim
Prenatal intervention of severe fetal aortic valve stenosis by ultrasound-guided percutaneous balloon valvuloplasty has been performed to prevent the progression to hypoplastic left heart syndrome, and achieve biventricular circulation in neonates. Here we report a case of fetal aortic valvuloplasty prenatally diagnosed with aortic stenosis at 24 weeks of gestation and showed worsening features on a follow-up echocardiography. Prenatal aortic valvuloplasty was performed at 29 weeks of gestation, and was a technical success...
January 2017: Obstetrics & Gynecology Science
https://www.readbyqxmd.com/read/28211263/increased-regurgitant-flow-causes-endocardial-cushion-defects-in-an-avian-embryonic-model-of-congenital-heart-disease
#2
Stephanie M Ford, Matthew T McPheeters, Yves T Wang, Pei Ma, Shi Gu, James Strainic, Christopher Snyder, Andrew M Rollins, Michiko Watanabe, Michael W Jenkins
BACKGROUND: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart...
February 17, 2017: Congenital Heart Disease
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
#3
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/28190605/the-importance-of-age-and-weight-on-cavopulmonary-shunt-stage-ii-outcomes-after-the-norwood-procedure-planned-versus-unplanned-surgery
#4
David J Barron, Intisar Ul Haq, Adrian Crucean, John Stickley, Phil Botha, Natasha Khan, Timothy J Jones, William J Brawn
OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit...
January 17, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28184977/lateral-atrial-tunnel-fontan-operation-predisposes-to-the-junctional-rhythm
#5
Katarzyna Januszewska, Anna Schuh, Anja Lehner, Robert Dalla-Pozza, Edward Malec
The goal of the study was to compare the early postoperative course after fenestrated lateral atrial tunnel (LT) and non-fenestrated extracardiac conduit (EC) Fontan operation (FO) in a single center where both techniques were parallelly used. Between 2004 and 2012, 56(32.7%) children underwent LT and 113(66.1%) EC FO. The mean age was 3.7 ± 2.9 years and mean weight was 14.6 ± 7.3 kg. The most common malformation was hypoplastic left heart syndrome (50.9%). The general approach was to perform LT in children after hemi-Fontan operation and EC in patients after Glenn anastomosis...
February 10, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28174392/-heart-transplantation-allograft-and-xenograft
#6
Norihide Fukushima
Prior to starting clinical cardiac allotransplantation, cardiac xenotransplantation was performed in human in 1960s. In 1964, Hardy performed cardiac transplantation using a chimpanzee heart and Bailey performed cardiac transplantation using a baboon heart to an infant with hypoplastic left heart. The use of cyclosporine has greatly improved the outcome of clinical cardiac transplantation and cardiac allotransplantation became an established treatment strategy for the patients with end-stage heart failure. Although concordant cardiac xenotransplantation from a primate to a human may be successfully performed using current immunosuppressive regimen, a primate heart is not a good candidate for cardiac xenograft due to animal light issues and its size...
January 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28169036/identification-of-risk-factors-for-poor-feeding-in-infants-with-congenital-heart-disease-and-a-novel-approach-to-improve-oral-feeding
#7
Gitanjali Indramohan, Tiffany P Pedigo, Nicole Rostoker, Mae Cambare, Tristan Grogan, Myke D Federman
Many infants with complex congenital heart disease (CHD) do not develop the skills to feed orally and are discharged home on gastrostomy tube or nasogastric feeds. We aimed to identify risk factors for failure to achieve full oral feeding and evaluate the efficacy of oral motor intervention for increasing the rate of discharge on full oral feeds by performing a prospective study in the neonatal and cardiac intensive care units of a tertiary children's hospital. 23 neonates born at ≥37weeks gestation and diagnosed with single-ventricle physiology requiring a surgical shunt were prospectively enrolled and received oral motor intervention therapy...
February 3, 2017: Journal of Pediatric Nursing
https://www.readbyqxmd.com/read/28148317/echocardiography-of-coarctation-of-the-aorta-aortic-arch-hypoplasia-and-arch-interruption-strategies-for-evaluation-of-the-aortic-arch
#8
REVIEW
Suma P Goudar, Sanket S Shah, Girish S Shirali
: Aim Echocardiography is the modality of choice for the diagnosis and serial follow-up of aortic arch pathology. In this article, we review the types of obstruction of the aortic arch, various classification schemes of coarctation of the aorta and interrupted aortic arch, methodology for optimal echocardiographic imaging of the aortic arch, and key echocardiographic measurements for accurate diagnosis of obstruction and hypoplasia of the aortic arch. Finally, we will discuss the limitations of echocardiography in optimal imaging of the aortic arch and the use of other non-invasive imaging modalities such as CT or MRI to provide additional information in these cases...
December 2016: Cardiology in the Young
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
#9
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/28132535/modified-total-cavopulmonary-shunt-as-a-staged-fontan-operation
#10
Hidetsugu Asai, Tsuyoshi Tachibana, Yasushige Shingu, Hiroki Kato, Satoru Wakasa, Yoshiro Matsui
The left superior vena cava became occluded in an infant with hypoplastic left heart syndrome. After a bidirectional Glenn procedure, he presented with severe oxygen desaturation and right ventricular dysfunction; the left superior vena cava drained into the inferior vena cava through collateral veins. As salvage therapy, we created a modified total cavopulmonary shunt using only autologous tissue in which the right hepatic vein and inferior vena cava drained into the pulmonary artery via a lateral tunnel in the right atrium...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
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
#11
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
#12
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/28112330/-surgical-site-infections-in-children-undergoing-cardiac-surgery-with-delayed-sternal-closure-case-control-study
#13
Javiera Retamal, Pedro Becker, Rodrigo González, Marcela Ferrés, Jaime Cerda, María I Riquelme, Regina Pérez, Cristián Clavería
INTRODUCTION: Surgical site infections (SSI) are an important cause of morbidity in pediatric cardiac surgery. Risk factors in patients requiring delayed sternal closure (DSC) are unknown. AIM: To report the rate of SSI in children undergoing cardiac surgery with DSC and determine the risk factors. METHODOLOGY: A retrospective case-control study, in patients younger than 15 years old undergoing cardiac surgery with DSC in our center between 2009 and 2010...
October 2016: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
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
#14
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/28093805/heartware-ventricular-assist-device-implantation-in-patients-with-fontan-physiology
#15
Bartlomiej R Imielski, Robert A Niebler, Steven J Kindel, Ronald K Woods
We aim to describe the clinical course of a series of patients with hypoplastic left heart syndrome and refractory systolic heart failure supported with a HeartWare ventricular assist device (HVAD) following Fontan palliation. This is a retrospective review of three consecutive patients supported with a HVAD following Fontan palliation through February 2016. Data include patient characteristics, operative variables, postimplantation hemodynamic/device parameters, event outcomes, and duration of HVAD support...
January 2017: Artificial Organs
https://www.readbyqxmd.com/read/28072476/aberrant-right-subclavian-artery-correlation-between-fetal-and-neonatal-abnormalities-and-abnormal-genetic-screening-or-testing
#16
Angela C Ranzini, Francine Hyman, Emily Jamaer, Tim van Mieghem
OBJECTIVES: To determine whether fetuses with an isolated aberrant course of the right subclavian artery (ARSA) have increased risk for chromosomal abnormalities, including trisomy 21 or 22q11 deletion. METHODS: We performed a retrospective chart review of all fetuses with antenatally diagnosed ARSA. Data were collected from fetal anatomic surveys, fetal echocardiograms, noninvasive trisomy 21 screening programs, invasive genetic studies, and neonatal records. RESULTS: Seventy-nine fetuses with ARSA were identified at 20...
January 10, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
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
#17
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/28043454/surgical-techniques-for-repair-of-peripheral-pulmonary-artery-stenosis
#18
Richard D Mainwaring, Frank L Hanley
Peripheral pulmonary artery stenosis (PPAS) is a rare form of congenital heart disease that is most frequently associated with Williams and Alagille syndromes. These patients typically have systemic level right ventricular pressures secondary to obstruction at the lobar, segmental, and subsegmental branches. The current management of patients with PPAS remains somewhat controversial. We have pioneered an entirely surgical approach for the reconstruction of PPAS. This approach initially entailed a surgical patch augmentation of all major lobar branches and effectively reduced the right ventricular pressures by more than half...
July 2016: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28033074/preoperative-feeding-neonates-with-cardiac-disease
#19
Carly J Scahill, Eric M Graham, Andrew M Atz, Scott M Bradley, Minoo N Kavarana, Sinai C Zyblewski
BACKGROUND: The potential for necrotizing enterocolitis (NEC) in neonates requiring cardiac surgery has contributed largely to wide feeding practice variations and a hesitation to initiate enteral feeding during the preoperative period, specifically those patients with hypoplastic left heart syndrome. METHODS: A retrospective chart review of neonates undergoing cardiac surgery at a single institution between July 2011 and July 2013 was performed. The primary objective of this study was to determine if preoperative feeding was associated with NEC in neonates requiring cardiac surgery...
January 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28024650/recoarctation-after-norwood-i-procedure-for-hypoplastic-left-heart-syndrome-impact-of-patch-material
#20
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
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