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https://www.readbyqxmd.com/read/29788322/outcomes-and-risk-factors-associated-with-tricuspid-valve-repair-in-children-with-hypoplastic-left-heart-syndrome
#1
Bahaaldin Alsoufi, Raina Sinha, Courtney McCracken, Janet Figueroa, Firat Altin, Kirk Kanter
OBJECTIVES: Tricuspid valve (TV) competence is important for successful palliation of hypoplastic left heart syndrome (HLHS). We report our experience with TV repair in HLHS patients with a focus on TV and right ventricular (RV) function and associated clinical outcomes. METHODS: From 2002 to 2012, 219 neonates with HLHS underwent the Norwood operation. Thirty patients who underwent TV repair at various stages comprised our current series cohort. Echocardiographic and clinical data were reviewed to determine the effectiveness of TV repair and outcomes of the patients...
May 19, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29777281/right-ventricular-systolic-function-parameters-in-hypoplastic-left-heart-syndrome
#2
S Javed Zaidi, Jamie Penk, Vivian W Cui, Supaluck Kanjanauthai, David A Roberson
Assessment of the systolic function of the right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS) is important. The asymmetric shape and heavy trabeculations make accurate assessment of RV systolic function challenging. Novel measures of RV function could be helpful in distinguishing reduced versus preserved function in HLHS and may also be worse in HLHS with preserved function compared to normal controls. These novel methods offer promise, but research and clinical applicability is hindered as no cut-off values for normal function have been established...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29774394/longitudinal-health-care-cost-in-hypoplastic-left-heart-syndrome-palliation
#3
Jesse E Hansen, Nicolas L Madsen, Laurie Bishop, David L S Morales, Jeffrey B Anderson
Management of hypoplastic left heart syndrome (HLHS) is resource intensive. Heath care systems are pressured to provide value to patients by improving outcomes while decreasing costs. A single-center retrospective cohort of infants with HLHS who underwent Norwood procedure or hybrid Norwood from 2004 to 2014 and survived to first outpatient follow up were studied. The primary outcome was total cost through 12 months with a sub-analysis of patients with 60 months of data. Costs were calculated using internal cost accounting system and reported by cost center...
May 17, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#4
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29754919/balloon-expandable-stents-for-recoarctation-of-the-aorta-in-small-children-two-centre-experience
#5
K Gendera, P Ewert, D Tanase, S Georgiev, T Genz, P Bambul Heck, T Moszura, I Malcic, J Cleuziou, A Eicken
BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents...
July 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29754202/regional-differences-in-cost-and-length-of-stay-in-neonates-with-hypoplastic-left-heart-syndrome
#6
Luma Essaid, Paula D Strassle, Eric G Jernigan, Jennifer S Nelson
Hypoplastic left heart syndrome (HLHS) is a highly resource-intensive diagnosis. Geographic variation in cost and length of stay (LOS) in HLHS is not well described. Neonates diagnosed with HLHS between 2000 and 2012 were identified using the Kids' Inpatient Database. Hospitalizations were stratified into two groups: (1) birth and (2) secondary. United States regional differences in hospital charges and LOS were compared using adjusted linear regression. Of 2431 birth hospitalizations, 449 neonates (18.5%) died while inpatient and mortality rates differed by region (p = 0...
May 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29748699/longitudinal-deformation-of-the-right-ventricle-in-hypoplastic-left-heart-syndrome-a-comparative-study-of-2d-feature-tracking-magnetic-resonance-imaging-and-2d-speckle-tracking-echocardiography
#7
Mona Salehi Ravesh, Carsten Rickers, Finn Jonathan Bannert, David Hautemann, Abdullah Al Bulushi, Dominik Daniel Gabbert, Philip Wegner, Eva Kis, Jan Hinnerk Hansen, Michaeal Jerosch-Herold, H-H Kramer, Jana Logoteta
In hypoplastic left heart syndrome (HLHS), long-term outcome is closely related to right ventricular function. Echocardiography and magnetic resonance imaging (MRI) are routinely used for functional assessment. MRI 2D-tissue feature tracking (2D-FT) allows quantification of myocardial deformation but has not yet been applied to HLHS patients. We sought to investigate the feasibility of this technique and to compare the results to 2D-speckle tracking echocardiography (2D-STE). In routine MRI 2D anatomical four chamber view, cine images were recorded in 55 HLHS patients (median age 4...
May 10, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29712484/does-prenatal-diagnosis-of-critical-congenital-heart-diseases-influence-the-prereferral-mortality-in-a-center-without-surgical-intervention
#8
Buse Özer Bekmez, Evrim Alyamaç Dizdar, Nilüfer Okur, Mehmet Büyüktiryaki, Nurdan Uraş, Serife Suna Oğuz
BACKGROUND: Prenatal diagnosis ameliorates some preoperative and postoperative outcomes in critical congenital heart disease (CHD). Despite large variability among anatomical defect types, nearly half of them are diagnosed antenatally. We aimed to investigate the effect of the antenatal diagnosis on prereferral mortality of infants with critical CHD in a center without cardiovascular surgery clinic. METHODS: Medical records of the neonates who were diagnosed with critical CHD between the years 2010 and 2016 in Zekai Tahir Burak Women's health Education and Research Hospital were retrospectively reviewed for the study...
April 30, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29683008/first-trimester-combined-screening-biochemistry-in-detection-of-congenital-heart-defects
#9
Julia Alanen, Teemu Korpimaki, Heikki Kouru, Mikko Sairanen, Markku Leskinen, Mika Gissler, Markku Ryynanen, Jaana Nevalainen
OBJECTIVE: To evaluate the performance of first trimester biochemical markers, pregnancy-associated plasma protein-A (PAPP-A), free beta human chorionic gonadotropin (fβ-hCG), and nuchal translucency (NT) in detection of severe congenital heart defects (CHDs). METHODS: During the study period from 1 January 2008 to 31 December 2011, biochemical markers and NT were measured in 31,144 women as part of voluntary first trimester screening program for Down's syndrome in Northern Finland...
April 22, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29664500/nutritional-recovery-after-cardiac-surgery-in-children-with-congenital-heart-disease
#10
I Oyarzún, C Claveria, G Larios, C Le Roy
INTRODUCTION: Malnutrition is common in children with congenital heart disease (CHD). Medical treatment and surgical interventions contribute improving the nutritional status of these children. OBJECTIVE: To describe nutritional recovery in children with CHD and associated factors after surgery. PATIENTS AND METHOD: Longitudinal study. 46 Children under 18 years old admitted for CHD surgery between April 2015 and April 2016 were recruited...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29580790/recognition-of-neonatal-lymphatic-flow-disorder-fetal-mr-findings-and-postnatal-mr-lymphangiogram-correlation
#11
David M Biko, Jordan A Johnstone, Yoav Dori, Teresa Victoria, Edward R Oliver, Maxim Itkin
RATIONALE AND OBJECTIVES: This study aimed to describe prenatal and postnatal imaging features and outcomes of neonates with neonatal lymphatic disorders (NLDs). MATERIALS AND METHODS: An institutional review board-approved search of the radiology database for patients with NLD identified five patients. Inclusion criteria include prenatal imaging (fetal magnetic resonance [MR] imaging and ultrasound) and postnatal three-dimensional T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) and dynamic contrast-enhanced MR lymphangiography within 6 months of life...
March 23, 2018: Academic Radiology
https://www.readbyqxmd.com/read/29577004/staged-surgical-palliation-for-hlhs-in-a-girl-with-severe-factor-x-deficiency
#12
Ahmed F Elmahrouk, Mohamed F Ismail, Abdulbadee Bugis, Nashwa Badawy, Hesham Mohamed Aboelghar, Tamer Hamouda, Ahmed Jamjoom
Background  Factor X deficiency (also known as Stuart-Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation  This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication...
January 2018: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/29571602/heart-failure-after-the-norwood-procedure-an-analysis-of-the-single-ventricle-reconstruction-trial
#13
William T Mahle, Chenwei Hu, Felicia Trachtenberg, JonDavid Menteer, Steven J Kindel, Anne I Dipchand, Marc E Richmond, Kevin P Daly, Heather T Henderson, Kimberly Y Lin, Michael McCulloch, Ashwin K Lal, Kurt R Schumacher, Jeffrey P Jacobs, Andrew M Atz, Chet R Villa, Kristin M Burns, Jane W Newburger
BACKGROUND: Heart failure results in significant morbidity and mortality in young children with hypoplastic left heart syndrome (HLHS) after the Norwood procedure. METHODS: We studied subjects enrolled in the prospective Single Ventricle Reconstruction (SVR) Trial who survived to hospital discharge after a Norwood operation and were followed up to age 6 years. The primary outcome was heart failure, defined as heart transplant listing after Norwood hospitalization, death attributable to heart failure, or symptomatic heart failure (New York Heart Association [NYHA] Class IV)...
February 16, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29569026/the-genetic-landscape-of-hypoplastic-left-heart-syndrome
#14
Hisato Yagi, Xiaoqin Liu, George C Gabriel, Yijen Wu, Kevin Peterson, Stephen A Murray, Bruce J Aronow, Lisa J Martin, D Woodrow Benson, Cecilia W Lo
Hypoplastic left heart syndrome (HLHS) is one of the most lethal congenital heart defects, and remains clinically challenging. While surgical palliation allows most HLHS patients to survive their critical heart disease with a single-ventricle physiology, many will suffer heart failure, requiring heart transplantation as the only therapeutic course. Current paradigm suggests HLHS is largely of hemodynamic origin, but recent findings from analysis of the first mouse model of HLHS showed intrinsic cardiomyocyte proliferation and differentiation defects underlying the left ventricular (LV) hypoplasia...
March 22, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29546398/novel-aortic-arch-reconstruction-using-a-modified-norwood-procedure-based-on-hypoplastic-left-heart-syndrome-specific-anatomical-malformations
#15
Takeshi Konuma, Syunsuke Sakamoto, Syuhei Toba, Ayano Futsuki, Naoki Yamamoto, Shinji Kanemitsu, Hideto Shimpo
OBJECTIVES: Postoperative left pulmonary artery (PA) or bronchus compression occasionally follows narrowing of the retroaortic space after the Norwood procedure. We examined hypoplastic left heart syndrome (HLHS)-specific anatomical malformations and hypothesized that a long main pulmonary arterial trunk might contribute to a larger retroaortic space. In this study, we introduced a modified Norwood procedure [pulmonary artery trunk-saving method (PATS)] and evaluated the results. METHODS: HLHS-specific anatomical malformations were examined using computed tomographic data from 23 consecutive patients who underwent bilateral pulmonary banding...
March 12, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29544421/early-outcomes-of-hypoplastic-left-heart-syndrome-infants-meta-analysis-of-studies-comparing-the-hybrid-and-norwood-procedures
#16
Jacob Y Cao, Seung Yeon Lee, Kevin Phan, Julian Ayer, David S Celermajer, David S Winlaw
The hybrid strategy is an alternative to the traditional Norwood procedure for initial palliation of infants with hypoplastic left heart syndrome (HLHS) who are deemed to be at high surgical risk. Numerous single-center studies have compared the two procedures, showing similar early outcomes, although the cohort sizes are likely insufficiently powered to detect significant differences. The current meta-analysis aims to explore the early morbidity and mortality associated with the hybrid compared to the Norwood procedure...
March 2018: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/29540791/different-vasodilatation-characteristics-among-the-main-cerebral-arteries-in-fetuses-with-congenital-heart-defects
#17
Qinghai Peng, Shi Zeng, Qichang Zhou, Wen Deng, Tao Wang, Ya Tan, Yushan Liu
To observe Doppler changes in the three main cerebral arteries in fetuses with congenital heart defects (CHDs). The pulsatility index (PI) values of the anterior cerebral artery (ACA), middle cerebral artery (MCA), and posterior cerebral artery (PCA) were prospectively compared in 78 CHD fetuses and 78 normal control fetuses. Correlations between the cerebral artery PIs and the neurodevelopment scores (psychomotor development index [PDI] and mental development index [MDI]) were assessed. The MCA-PI was decreased significantly in fetuses with hypoplastic left heart syndrome (HLHS)...
March 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29524308/differences-in-midterm-outcomes-in-infants-with-hypoplastic-left-heart-syndrome-diagnosed-with-necrotizing-enterocolitis-npcqic-database-analysis
#18
Natasha L Lopez, Charitha Gowda, Carl H Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L Cua
INTRODUCTION: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period. METHODS: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed...
March 9, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29521004/delayed-puberty-and-abnormal-anthropometry-and-its-associations-with-quality-of-life-in-young-fontan-survivors-a-multicenter-cross-sectional-study
#19
Shaji C Menon, Ragheed Al-Dulaimi, Brian W McCrindle, David J Goldberg, Ritu Sachdeva, Bryan H Goldstein, Thomas Seery, Karen C Uzark, Anjali Chelliah, Ryan Butts, Heather Henderson, Tiffanie Johnson, Richard V Williams
INTRODUCTION: We sought to evaluate the prevalence of delayed puberty and abnormal anthropometry and its association with quality of life (QoL) in young Fontan survivors. METHODS: This was a cross-sectional study at 11 Pediatric Heart Network centers. Demographic and clinical data, anthropomety, and Tanner stage were collected. Anthropometric measurements and pubertal stage were compared to US norms. QoL was assessed using Pediatric Quality of Life inventory (PedsQL)...
March 9, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29497771/cerebral-blood-flow-following-hybrid-stage-i-palliation-in-infants-with-hypoplastic-left-heart-syndrome
#20
Sharon L Cheatham, Joanne L Chisolm, Nicole O'Brien
Hypoplastic left heart syndrome (HLHS) palliation may result in altered cerebral blood flow with subsequent neurodevelopmental implications. The purpose of the study was to assess blood flow in the middle cerebral artery (MCA) and investigate the relationship with early neurodevelopmental outcomes in infants with HLHS after hybrid stage I. Transcranial Doppler (TCD) was performed to obtain peak systolic, end-diastolic, and mean velocities, as well as pulsatility index of the MCA in infants with HLHS (n = 18) at baseline and at 2, 4, and 6 months of age...
April 2018: Pediatric Cardiology
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