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Hypoplastic left heart

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https://www.readbyqxmd.com/read/27908945/norwood-procedure-for-palliation-of-hypoplastic-left-heart-syndrome-right-ventricle-to-pulmonary-artery-conduit-vs-modified-blalock-taussig-shunt
#1
Dorothy M Beke
Patients with hypoplastic left heart syndrome undergo a series of operations to separate the pulmonary and systemic circulations. The first of at least 3 operations occurs in the newborn period, with a stage I palliation. The goal of stage I palliation is to provide pulmonary blood flow and create an unobstructed systemic outflow tract. Advances in surgical techniques and intraoperative and postoperative care have helped decrease morbidity and mortality for patients with hypoplastic left heart syndrome who have the stage I Norwood operation, but the patients continue to be at increased risk for hemodynamic collapse and adverse outcomes...
December 2016: Critical Care Nurse
https://www.readbyqxmd.com/read/27896819/extracorporeal-membrane-oxygenation-outcomes-after-the-comprehensive-stage-ii-procedure-in-patients-with-single-ventricles
#2
Daniel Gomez, Vicky Duffy, Diane Hersey, Carl Backes, Peter Rycus, Patrick McConnell, Jordan Voss, Mark Galantowicz, Clifford L Cua
Outcomes for extracorporeal membrane oxygenation (ECMO) have been described for patients with single ventricle physiology (SVP) undergoing cavopulmonary connection (Glenn procedure). An alternative surgical pathway for patients with SVP consists of an initial hybrid procedure followed by a comprehensive Stage II procedure. No data exist describing the outcomes of patients requiring ECMO after the comprehensive Stage II procedure. The goal of this study is to describe the outcomes for patients who required ECMO after the comprehensive Stage II procedure...
November 29, 2016: Artificial Organs
https://www.readbyqxmd.com/read/27894567/proportion-of-selected-congenital-heart-defects-attributable-to-recognized-risk-factors
#3
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/27891328/abnormal-course-of-right-coronary-artery-together-with-variant-left-coronary-artery-and-partial-atresia-of-cicumflex-artery
#4
Rajani Singh
Heart is supplied by right and left coronary arteries. Therefore, the knowledge of normal and variant anatomy of both these arteries and their configurations is indispensable for proper understanding of diagnosis and treatment of dreaded cardiac diseases. During routine cadaveric dissection, a heart of an adult female with anomalous course of Right Coronary Artery (RCA), new variant of trifurcated Left Coronary Artery (LCA) and partial atresia of circumflex (CX) artery was detected. Trifurcation of LCA into anterior interventricular, CX and a ramus intermedius without giving accessory branches has been reported...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
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
#5
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/27879470/incidence-and-management-of-thrombotic-and-thromboembolic-complications-following-the-norwood-procedure-a-systematic-review
#6
Arnav Agarwal, Mohammed Firdouse, Nishaan Brar, Andy Yang, Panos Lambiris, Anthony K Chan, Tapas K Mondal
BACKGROUND: The stage 1 Norwood procedure and its variants represent the first step of palliation for hypoplastic left heart syndrome. Although appropriate postoperative thromboprophylaxis is integral, significant variance remains across institutional practices. The purpose of this systematic review is to estimate the incidence of thrombosis and thromboembolism following the Norwood or modified Blalock-Taussig shunt procedure and examine current thromboprophylaxis regimens. METHODS: Ovid MEDLINE and Embase were searched from January 2000 to June 2016 for primary studies explicitly reporting incidence of thrombosis, thromboembolism (strokes and pulmonary embolisms), or shunt occlusion in neonates, infants, and children undergoing the Norwood procedure or any variant...
November 21, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27865477/tracheostomy-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-cardiac-operations-a-multicenter-analysis
#7
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/27860551/congenital-variants-and-anomalies-of-the-aortic-arch
#8
Kate Hanneman, Beverley Newman, Frandics Chan
Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation...
November 18, 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/27855999/assessment-of-growth-6-years-after-the-norwood-procedure
#9
Phillip T Burch, Chitra Ravishankar, Jane W Newburger, Linda M Lambert, Victoria L Pemberton, Suzanne Granger, Alejandro A Floh, Jeffrey B Anderson, Garick D Hill, Kevin D Hill, Matthew E Oster, Alan B Lewis, Kurt R Schumacher, Sinai C Zyblewski, Ryan R Davies, Jeffrey P Jacobs, Wyman W Lai, L LuAnn Minich
: At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00115934.
November 14, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27850459/821-familial-impact-and-coping-in-parents-of-children-with-hypoplastic-left-heart-syndrome
#10
Agustin Cabrera, Elena Ocampo, Valeria Munguia
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27821206/the-neurodevelopmental-implications-of-hypoplastic-left-heart-syndrome-in-the-fetus
#11
David F A Lloyd, Mary A Rutherford, John M Simpson, Reza Razavi
As survival after cardiac surgery continues to improve, an increasing number of patients with hypoplastic left heart syndrome are reaching school age and beyond, with growing recognition of the wide range of neurodevelopmental challenges many survivors face. Improvements in fetal detection rates, coupled with advances in fetal ultrasound and MRI imaging, are contributing to a growing body of evidence that abnormal brain architecture is in fact present before birth in hypoplastic left heart syndrome patients, rather than being solely attributable to postnatal factors...
November 8, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27821007/infant-health-after-heart-surgery
#12
(no author information available yet)
Background The number of infants who survive initial surgery for complex congenital heart disease (CHD), such as hypoplastic left heart syndrome, is increasing, but they are often left with residual complex health needs.
November 8, 2016: Nursing Children and Young People
https://www.readbyqxmd.com/read/27817953/mechanically-assisted-bidirectional-cavopulmonary-shunt-in-neonates-and-infants-an-acute-human-pilot-study
#13
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
#14
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...
October 29, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27803958/predicting-the-need-for-neoaortic-arch-intervention-in-infants-with-hypoplastic-left-heart-syndrome-through-the-glenn-procedure
#15
Mamata Eagam, Rohit S Loomba, Andrew N Pelech, James S Tweddell, Edward Kirkpatrick
Neoaortic recoarctation is present in up to over one-third of patients having undergone the Norwood procedure for hypoplastic left heart syndrome. Some of these patients will require reintervention by catheterization or surgery through the time of the Glenn procedure. Echocardiography and catheterization are often utilized in this period to assess hemodynamics although no specific criteria have been identified to predict whether and when neoaortic arch reintervention will be needed. We sought to identify predictors, including but not limited to echocardiographic and catheterization gradients, to predict such intervention...
November 1, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27803957/interstage-survival-for-patients-with-hypoplastic-left-heart-syndrome-after-ecmo
#16
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
#17
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
#18
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/27777296/current-therapy-for-hypoplastic-left-heart-syndrome-and-related-single-ventricle-lesions
#19
Richard G Ohye, Dietmar Schranz, Yves D'Udekem
Universally fatal only 4 decades ago, the progress in the 3-stage palliation of hypoplastic left heart syndrome and related single right ventricular lesions has drastically improved the outlook for these patients. Although the stage II operation (hemi-Fontan or bidirectional Glenn) and stage III Fontan procedure have evolved into relatively low-risk operations, the stage I Norwood procedure remains one of the highest-risk and costliest common operations performed in congenital heart surgery. Yet, despite this fact, experienced centers now report hospital survivals of >90% for the Norwood procedure...
October 25, 2016: Circulation
https://www.readbyqxmd.com/read/27768247/associations-between-maternal-water-consumption-and-birth-defects-in-the-national-birth-defects-prevention-study-2000-2005
#20
Breanna L Alman, Evan Coffman, Anna Maria Siega-Riz, Thomas J Luben
BACKGROUND: Water and water-based beverages constitute a major part of daily fluid intake for pregnant women, yet few epidemiologic studies have investigated the role of water consumption on birth outcomes. METHODS: We used data from the National Birth Defects Prevention Study to conduct a case-control study investigating associations between maternal water consumption during pregnancy and birth defects (BD). We used interview data on water consumption during the first trimester of pregnancy in 14,454 cases (major BDs n ≥ 50) and 5,063 controls...
October 21, 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
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