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Hypoplastic left heart

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https://www.readbyqxmd.com/read/29664500/nutritional-recovery-after-cardiac-surgery-in-children-with-congenital-heart-disease
#1
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/29655509/reduced-right-ventricular-fractional-area-change-strain-and-strain-rate-before-bidirectional-cavopulmonary-anastomosis-is-associated-with-medium-term-mortality-for-children-with-hypoplastic-left-heart-syndrome
#2
Lily Q Lin, Jennifer Conway, Silvia Alvarez, Benjamin Goot, Jesus Serrano-Lomelin, Timothy Colen, Edythe B Tham, Shelby Kutty, Ling Li, Nee Scze Khoo
BACKGROUND: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). METHODS: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery...
April 12, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29649824/end-organ-saturation-differences-in-early-neonatal-transition-for-left-versus-right-sided-congenital-heart-disease
#3
Gabriel Altit, Shazia Bhombal, Theresa A Tacy, Valerie Y Chock
BACKGROUND: For neonates with congenital heart disease (CHD), left-sided (LL) and right-sided (RL) single ventricular physiologies (LL, hypoplastic left heart syndrome; RL, tricuspid atresia or pulmonary atresia with intact ventricular septum) may demonstrate distinct changes in tissue saturation in the first 72 h of life. Near-infrared spectroscopy (NIRS) can measure regional cerebral saturation (Csat) and renal saturation (Rsat) to clarify differences between LL and RL over time. OBJECTIVES: Our primary objective was to measure changes in Csat and Rsat in the first 72 h of life using NIRS between CHD infants with LL compared to RL...
April 12, 2018: Neonatology
https://www.readbyqxmd.com/read/29609227/-characteristics-and-prognosis-of-interrupted-inferior-vena-cava-with-azygous-continuation
#4
Q Xu, H X Sun, J S Xie, J L Wang, Q Y Pei, X H Zhang
Objective: To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Methods: Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan. The clinical data, ultrasonographic features, genetic information and prognosis were collected. Results: Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567) of 28 567 patients...
March 25, 2018: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/29580790/recognition-of-neonatal-lymphatic-flow-disorder-fetal-mr-findings-and-postnatal-mr-lymphangiogram-correlation
#5
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
#6
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
#7
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
#8
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/29549184/congenital-left-sided-heart-obstruction
#9
REVIEW
Michelle Carr, Stephanie Curtis, Jan Marek
Congenital obstruction of the left ventricular outflow tract remains a significant problem and multi-level obstruction can often co-exist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation...
March 16, 2018: Echo Research and Practice
https://www.readbyqxmd.com/read/29549091/hypoplastic-left-heart-the-next-chapter-in-our-unsolved-problem
#10
EDITORIAL
David S Winlaw
No abstract text is available yet for this article.
March 16, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29546398/novel-aortic-arch-reconstruction-using-a-modified-norwood-procedure-based-on-hypoplastic-left-heart-syndrome-specific-anatomical-malformations
#11
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
#12
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
#13
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/29534193/modified-ross-konno-procedure-in-children-subcoronary-implantation-technique-with-konno-incision-for-annular-and-subannular-hypoplasia
#14
Peter Murin, Nicodème Sinzobahamvya, Antonia Schulz, Victoria Lorenzen, Stanislav Ovroutski, Felix Berger, Joachim Photiadis, Mi-Young Cho
OBJECTIVES: The technique of subcoronary autograft implantation for the Ross procedure has shown excellent durability of aortic valve function in adults. However, its use in children with hypoplastic aortic annulus or multilevel left ventricular outflow tract obstruction (LVOTO) was traditionally precluded. We combined this technique with a Konno incision and evaluated LVOTO relief and durability of the autograft function in growing patients. METHODS: Between January 2012 and January 2017, 13 patients with severe LVOTO and hypoplastic aortic annulus underwent Ross-Konno procedure with subcoronary autograft implantation...
March 9, 2018: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/29526349/necrotizing-enterocolitis-in-patients-with-congenital-heart-disease-a-single-center-experience
#15
Patricio E Lau, Stephanie M Cruz, Elena C Ocampo, Sushma Nuthakki, Candace C Style, Timothy C Lee, David E Wesson, Oluyinka O Olutoye
PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation...
February 7, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29524308/differences-in-midterm-outcomes-in-infants-with-hypoplastic-left-heart-syndrome-diagnosed-with-necrotizing-enterocolitis-npcqic-database-analysis
#16
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/29523922/a-novel-mechanism-for-improved-exercise-performance-in-pediatric-fontan-patients-after-cardiac-rehabilitation
#17
Samuel Wittekind, Wayne Mays, Yvette Gerdes, Sandra Knecht, John Hambrook, William Border, John Lynn Jefferies
Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. In this prospective study, pediatric Fontans completed two 60 min CR sessions per week for 12 weeks. Cardiopulmonary exercise testing and stress echocardiography were performed at baseline and last CR session, and then compared with a paired sample t test...
March 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29523921/impact-of-hybrid-stage-1-palliation-for-hypoplastic-left-heart-syndrome-histopathological-findings
#18
Takashi Kido, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Kenichi Kurosaki, Hatsue Ishibashi-Ueda, Hajime Ichikawa
The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared...
March 9, 2018: Pediatric Cardiology
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/29510943/laryngeal-ultrasound-detects-a-high-incidence-of-vocal-cord-paresis-after-aortic-arch-repair-in-neonates-and-young-children
#20
Melissa G Y Lee, Johnny Millar, Elizabeth Rose, Aleesha Jones, Dora Wood, Taryn L Luitingh, Diana Zannino, Johann Brink, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
OBJECTIVES: To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy. METHODS: Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation...
February 9, 2018: Journal of Thoracic and Cardiovascular Surgery
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