hypoplastic left heart syndrome

Summary

Summary: A condition characterized by underdevelopment of the left cardiac chambers, atresia or stenosis of the aorta or mitral valve or both, and hypoplasia of the aorta. These anomalies are a common cause of heart failure in early infancy.

Top Publications

  1. ncbi Hypoplastic left heart syndrome is heritable
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Am Coll Cardiol 50:1590-5. 2007
  2. ncbi Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis
    Ra K Han
    Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
    J Am Coll Cardiol 50:2406-14. 2007
  3. ncbi Computational hemodynamic analysis in congenital heart disease: simulation of the Norwood procedure
    Y Qian
    Center for Advanced Biomedical Science, TWIns, Waseda University, Tokyo, Japan
    Ann Biomed Eng 38:2302-13. 2010
  4. ncbi Maladaptive aortic properties in children after palliation of hypoplastic left heart syndrome assessed by cardiovascular magnetic resonance imaging
    Inga Voges
    Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig Holstein, Kiel, Germany
    Circulation 122:1068-76. 2010
  5. ncbi Identification of connexin43 (alpha1) gap junction gene mutations in patients with hypoplastic left heart syndrome by denaturing gradient gel electrophoresis (DGGE)
    C Dasgupta
    Department of Biochemistry, School of Medicine, Loma Linda University, Loma Linda, CA 92354, USA
    Mutat Res 479:173-86. 2001
  6. ncbi Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants
    David A Hehir
    Division of Cardiology, Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Thorac Cardiovasc Surg 136:94-9, 99.e1-3. 2008
  7. ncbi Identification of de novo mutations and rare variants in hypoplastic left heart syndrome
    M Iascone
    Laboratorio di Genetica Medica, Ospedali Riuniti, Bergamo, Italy
    Clin Genet 81:542-54. 2012
  8. ncbi Hypoplastic left heart syndrome: conventional surgical management
    Edward L Bove
    Section of Cardiac Surgery, Department of Surgery, The University of Michigan School of Medicine, Ann Arbor, MI, USA
    Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 7:3-10. 2004
  9. ncbi Changes of right ventricular function and longitudinal deformation in children with hypoplastic left heart syndrome before and after the Norwood operation
    Colin Petko
    Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig Holstein, Campus Kiel, Kiel, Germany
    J Am Soc Echocardiogr 24:1226-32. 2011
  10. ncbi Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome
    Vivek Muthurangu
    Cardiac MR Research Group, Division of Imaging Sciences, King s College London, United Kingdom
    Circulation 112:3256-63. 2005

Detail Information

Publications240 found, 100 shown here

  1. ncbi Hypoplastic left heart syndrome is heritable
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    J Am Coll Cardiol 50:1590-5. 2007
    This study sought to determine the size of the genetic effect (heritability) in families identified by a hypoplastic left heart syndrome (HLHS) proband.
  2. ncbi Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis
    Ra K Han
    Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
    J Am Coll Cardiol 50:2406-14. 2007
    ..The purpose of this study was to determine trends of growth of left heart structures after intervention for neonatal aortic valve stenosis...
  3. ncbi Computational hemodynamic analysis in congenital heart disease: simulation of the Norwood procedure
    Y Qian
    Center for Advanced Biomedical Science, TWIns, Waseda University, Tokyo, Japan
    Ann Biomed Eng 38:2302-13. 2010
    b>Hypoplastic left heart syndrome (HLHS) is a congenital heart disease which should be treated at neonate. Even now, its operation is one of the greatest challenges...
  4. ncbi Maladaptive aortic properties in children after palliation of hypoplastic left heart syndrome assessed by cardiovascular magnetic resonance imaging
    Inga Voges
    Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig Holstein, Kiel, Germany
    Circulation 122:1068-76. 2010
    The status of the reconstructed aorta in hypoplastic left heart syndrome is considered an important determinant of long-term prognosis...
  5. ncbi Identification of connexin43 (alpha1) gap junction gene mutations in patients with hypoplastic left heart syndrome by denaturing gradient gel electrophoresis (DGGE)
    C Dasgupta
    Department of Biochemistry, School of Medicine, Loma Linda University, Loma Linda, CA 92354, USA
    Mutat Res 479:173-86. 2001
    ..In the latter group, 14 children had hypoplastic left heart syndrome (HLHS) in which connexin43 gene defects were detected in eight...
  6. ncbi Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants
    David A Hehir
    Division of Cardiology, Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Thorac Cardiovasc Surg 136:94-9, 99.e1-3. 2008
    ..The purpose of this study is to identify risk factors for interstage death and to describe the events leading to interstage death...
  7. ncbi Identification of de novo mutations and rare variants in hypoplastic left heart syndrome
    M Iascone
    Laboratorio di Genetica Medica, Ospedali Riuniti, Bergamo, Italy
    Clin Genet 81:542-54. 2012
    b>Hypoplastic left heart syndrome (HLHS) is one of the most severe congenital heart malformations, characterized by underdevelopment of the structures in the left heart-aorta complex. The majority of cases are sporadic...
  8. ncbi Hypoplastic left heart syndrome: conventional surgical management
    Edward L Bove
    Section of Cardiac Surgery, Department of Surgery, The University of Michigan School of Medicine, Ann Arbor, MI, USA
    Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 7:3-10. 2004
    Surgical palliation for hypoplastic left heart syndrome has become standard therapy in many centers throughout the world...
  9. ncbi Changes of right ventricular function and longitudinal deformation in children with hypoplastic left heart syndrome before and after the Norwood operation
    Colin Petko
    Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital of Schleswig Holstein, Campus Kiel, Kiel, Germany
    J Am Soc Echocardiogr 24:1226-32. 2011
    ..in right ventricular (RV) function and deformation parameters before and at steady state after the Norwood operation in neonates with hypoplastic left heart syndrome. A further aim was to delineate factors that affected these changes.
  10. ncbi Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome
    Vivek Muthurangu
    Cardiac MR Research Group, Division of Imaging Sciences, King s College London, United Kingdom
    Circulation 112:3256-63. 2005
    ..In this study, we describe the results of 37 patients who underwent cardiovascular magnetic resonance (MR) assessment before BCPC...
  11. ncbi Cardiac homeobox gene NKX2-5 mutations and congenital heart disease: associations with atrial septal defect and hypoplastic left heart syndrome
    David A Elliott
    Victor Chang Cardiac Research Institute, 384 Victoria Street, Darlinghurst 2010, Sydney, Australia
    J Am Coll Cardiol 41:2072-6. 2003
    We sought to examine the importance of mutations in the cardiac transcription factor gene NKX2-5 in patients with an atrial septal defect (ASD), patent foramen ovale (PFO), or hypoplastic left heart syndrome (HLHS).
  12. ncbi Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988-1997
    Ruey Kang R Chang
    Division of Cardiology, Department of Pediatrics, Harbor UCLA Medical Center, Torrance, California 90509, USA
    Pediatrics 110:292-8. 2002
    To evaluate changes in the clinical management of infants with hypoplastic left heart syndrome (HLHS) over a 10-year period.
  13. ncbi Hypoplastic left heart syndrome
    David J Barron
    Department of Cardiac Surgery, Birmingham Children s Hospital, Birmingham, UK
    Lancet 374:551-64. 2009
    b>Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is underdeveloped...
  14. pmc Hypoplastic left heart syndrome links to chromosomes 10q and 6q and is genetically related to bicuspid aortic valve
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229 3039, USA
    J Am Coll Cardiol 53:1065-71. 2009
    This study was designed to identify disease loci for hypoplastic left heart syndrome (HLHS) and evaluate the genetic relationship between HLHS and bicuspid aortic valve (BAV).
  15. ncbi Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum
    Audrey C Marshall
    Department of Cardiology, Children s Hospital and Pediatrics, Harvard Medical School, 300 Longwood Ave, Boston, Mass 02115, USA
    Circulation 110:253-8. 2004
    Infants born with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum face a neonatal mortality of at least 48% despite early postnatal left atrial decompression and palliative surgery...
  16. ncbi Computational fluid dynamics in the evaluation of hemodynamic performance of cavopulmonary connections after the Norwood procedure for hypoplastic left heart syndrome
    Edward L Bove
    Section of Cardiac Surgery, Department of Surgery, University of Michigan School of Medicine, Ann Arbor, Mich, USA
    J Thorac Cardiovasc Surg 126:1040-7. 2003
    ..The purpose of this study was to compare hydraulic performance between the hemi-Fontan and bidirectional Glenn procedures, as well as the various types of completion Fontan operations...
  17. ncbi Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome
    Alexander A Kon
    Section of Pediatric Critical Care Medicine, University of California Davis, Ticon II, Room 228, 2516 Stockton Boulevard, Sacramento, CA 95817, USA
    Am J Cardiol 91:1506-9, A9. 2003
  18. pmc Prenatal head growth and white matter injury in hypoplastic left heart syndrome
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Pediatr Res 64:364-9. 2008
    Children with hypoplastic left heart syndrome (HLHS) have an increased prevalence of central nervous system (CNS) abnormalities...
  19. ncbi Fatal presentation of congenital isolated left ventricular apical hypoplasia
    Claire A Irving
    Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, England, UK
    Eur J Cardiothorac Surg 35:368-9. 2009
    ..We describe a sudden and fatal presentation of this anomaly in a previously well 19-year-old male, confirming the fact that this is not a benign condition but a spectrum with the potential for significant complications...
  20. ncbi The systolic to diastolic duration ratio in children with hypoplastic left heart syndrome: a novel Doppler index of right ventricular function
    Mark K Friedberg
    Division of Pediatric Cardiology, Department of Pediatrics, Lucille Packard Children s Hospital and Stanford University, Stanford, California, USA
    J Am Soc Echocardiogr 20:749-55. 2007
    Right ventricular (RV) function is an important determinant of clinical status in children with hypoplastic left heart syndrome (HLHS). However, assessment of RV function remains challenging because of its complex morphology...
  21. pmc An induced pluripotent stem cell model of hypoplastic left heart syndrome (HLHS) reveals multiple expression and functional differences in HLHS-derived cardiac myocytes
    Yan Jiang
    Institute of Genetic Medicine and Institute of Neuroscience, Newcastle University, Newcastle upon Tyne, United Kingdom Soochow University, Su Zhou, China Princess Al Jawhara Center of Excellence in Research, King Abdulaziz University, Jeddah, Saudi Arabia Centre for Biomedical Image Analysis, Faculty of Informatics, Masaryk University, Brno, Czech Republic Institute of Cardiovascular Science, Manchester Academic Health Science Centre, CoreTechnology Facility, Manchester, United Kingdom School of Biomedical Sciences, University of Durham, Durham, United Kingdom Department of Human Genetics, University of Kragujevac, Kragujevac, Serbia
    Stem Cells Transl Med 3:416-23. 2014
    b>Hypoplastic left heart syndrome (HLHS) is a serious congenital cardiovascular malformation resulting in hypoplasia or atresia of the left ventricle, ascending aorta, and aortic and mitral valves...
  22. ncbi Reversible ductus arteriosus constriction due to maternal indomethacin after fetal intervention for hypoplastic left heart syndrome with intact/restrictive atrial septum
    Melanie Vogel
    Department of Cardiology, Children s Hospital Boston, and Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    Fetal Diagn Ther 27:40-5. 2010
    ..Indomethacin promotes closure of the DA in newborns and in fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease...
  23. ncbi Impact of re-coarctation following the Norwood operation on survival in the balloon angioplasty era
    Ilana Zeltser
    Division of Cardiology, The Children s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    J Am Coll Cardiol 45:1844-8. 2005
    ..The objective of this study was to determine the efficacy of balloon angioplasty (BA) by comparing the immediate and long-term outcomes of patients with and without re-coarctation after a Norwood procedure...
  24. pmc Isolated left ventricular apical hypoplasia: a new congenital anomaly described with cardiac tomography
    M Fernandez-Valls
    Department of Radiology Section of Cardiovascular Imaging, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    Heart 90:552-5. 2004
    ..To describe cardiac tomography findings of an apparently new, presumably congenital, left ventricular (LV) abnormality noted consistently in three patients...
  25. ncbi Current issues and perspectives in hypoplasia of the left heart
    David Sedmera
    Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Cardiol Young 15:56-72. 2005
    b>Hypoplastic left heart syndrome is a rare but serious form of congenital cardiac disease, characterized by underdevelopment of the components of the left heart, rendering the left ventricle non-functional...
  26. ncbi Differential changes in TGF-β/BMP signaling pathway in the right ventricular myocardium of newborns with hypoplastic left heart syndrome
    Marco Ricci
    Division of Cardiothoracic Surgery, University of Miami Miller School of Medicine and Holtz Children s Hospital, Miami, FL, USA
    J Card Fail 16:628-34. 2010
    b>Hypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the left ventricle (LV) and increased biomechanical stress on the right ventricle (RV) from single ventricle physiology...
  27. pmc Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999-2001
    Kim L McBride
    Department of Molecular and Human Genetics, Columbus Children s Research Institute, Ohio State University, 43205, USA
    Birth Defects Res A Clin Mol Teratol 73:555-61. 2005
    ..outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) contribute significantly to infant mortality due to birth defects...
  28. ncbi Hypoplastic left heart syndrome: Rheumatic heart disease of the fetus?
    Pirooz Eghtesady
    Division of Paediatric Cardiothoracic Surgery, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
    Med Hypotheses 66:554-65. 2006
    b>Hypoplastic left heart syndrome (HLHS) accounts for nearly 25% of deaths among neonates with congenital heart disease. The essential feature of HLHS is a small left ventricle (LV) incapable of supporting the circulation...
  29. ncbi Hypoplastic left heart syndrome: current considerations and expectations
    Jeffrey A Feinstein
    Department of Pediatrics, Stanford University School of Medicine, Lucile Salter Packard Children s Hospital, Palo Alto, California 94304, USA
    J Am Coll Cardiol 59:S1-42. 2012
    ..defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS)...
  30. ncbi Increased incidence of Hirschsprung's disease in patients with hypoplastic left heart syndrome--a common neural crest-derived etiology?
    Juho Antti Ahola
    Department of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
    J Pediatr Surg 44:1396-400. 2009
    ..We hypothesize that the incidence of HD and bowel disorders associated with HD are increased in patients with hypoplastic left heart syndrome (HLHS) including left cardiac outflow tract obstruction.
  31. ncbi Isolated left-ventricular apical hypoplasia presenting as a left-ventricular mass on echocardiography
    Ching Ching Ong
    Department of Diagnostic Imaging, National University Hospital, 1E Kent Ridge Road, Tower Block, Level 12, Singapore, 119228, Singapore
    Pediatr Cardiol 33:1456-7. 2012
    ..This report describes an asymptomatic pediatric patient who initially presented with a left-ventricular mass on echocardiography...
  32. ncbi A plump and fatty heart: isolated left ventricular apical hypoplasia
    Kristine D Tumabiene
    Section of Cardiology, University of the Philippines Philippine General Hospital UP PGH, Manila, Philippines
    Echocardiography 29:E193-6. 2012
    ..To our knowledge, this is the first reported adult case in Asia...
  33. pmc Factors associated with subsequent arch reintervention after initial balloon aortoplasty in patients with Norwood procedure and arch obstruction
    Diego Porras
    Department of Cardiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    J Am Coll Cardiol 58:868-76. 2011
    ....
  34. ncbi Right ventricular function in fetal hypoplastic left heart syndrome
    Paul A Brooks
    Fetal and Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Women and Children s Health Research and Mazankowski Alberta Heart Institutes, University of Alberta, Edmonton, AB, Canada
    J Am Soc Echocardiogr 25:1068-74. 2012
    The systemic right ventricle in palliated hypoplastic left heart syndrome (HLHS) has relatively reduced longitudinal compared with circumferential deformation, a pattern of contraction more akin to the normal left ventricle, which ..
  35. ncbi Risk factors for profuse systemic-to-pulmonary artery collateral burden in hypoplastic left heart syndrome
    Ashwin Prakash
    Department of Cardiology, Boston Children s Hospital, Boston, MA, USA
    Am J Cardiol 112:400-4. 2013
    Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown...
  36. ncbi Superior hydrodynamics of a modified cavopulmonary connection for the Norwood operation
    H H Sievers
    Department of Cardiac Surgery, Medical University of Lubeck, Germany
    Ann Thorac Surg 65:1741-5. 1998
    ..Our hypothesis was that a modification of the standard Norwood variant of cavopulmonary connection with an extended anastomosis would improve hydrodynamics...
  37. ncbi Echocardiographic characterization of left ventricular apical hypoplasia accompanied by a patent ductus arteriosus
    Jessica A Haffajee
    Cardiovascular Imaging and Hemodynamic Laboratory, Tufts Medical Center, Boston 02111 MA, USA
    Eur J Echocardiogr 12:E17. 2011
    ..As more cases are recognized and patients are followed over time, the natural history and optimal treatment for this cardiomyopathy may be further elucidated...
  38. ncbi In-hospital mortality for children with hypoplastic left heart syndrome after stage I surgical palliation: teaching versus nonteaching hospitals
    Jay G Berry
    Pediatric Health Services Research Fellowship Program, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    Pediatrics 117:1307-13. 2006
    ..A substantial proportion of stage I palliation for hypoplastic left heart syndrome (HLHS) may be performed in nonteaching hospitals...
  39. ncbi Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring, and the results of early cavopulmonary anastomosis
    Nancy S Ghanayem
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
    Cardiol Young 16:61-6. 2006
  40. pmc Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome
    Anita Szwast
    The Fetal Heart Program at Cardiac Center at The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Circ Cardiovasc Imaging 3:172-8. 2010
    Cardiopulmonary interactions play an important role in the pathophysiology of hypoplastic left heart syndrome (HLHS). Pulmonary vasculopathy has been identified, especially in those with restrictive/intact atrial septum...
  41. ncbi The lateral tunnel Fontan procedure for hypoplastic left heart syndrome: results of 100 consecutive patients
    J C Hirsch
    Section of Cardiac Surgery, Division of Pediatric Cardiovascular Surgery, University of Michigan School of Medicine, 1500 E Medical Center Drive, Mott F7380, Ann Arbor, MI 48109 0223, USA
    Pediatr Cardiol 28:426-32. 2007
    The Fontan procedure for hypoplastic left heart syndrome (HLHS) is well established. Multiple surgical techniques including extracardiac conduits and autologous tissue connections have been developed...
  42. ncbi Real-time 3-dimensional echocardiography provides new insight into mechanisms of tricuspid valve regurgitation in patients with hypoplastic left heart syndrome
    K Takahashi
    Department of Pediatrics, University of Alberta, Alberta, Canada
    Circulation 120:1091-8. 2009
    Tricuspid regurgitation in hypoplastic left heart syndrome has an impact on outcome, but its mechanisms remain unclear.
  43. ncbi Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study
    Kartik S Sundareswaran
    Wallace H Coulter Department of Biomedical Engineering, Georgia Institute of Technology, Atlanta, Georgia 30332 0535, USA
    Ann Thorac Surg 82:1267-75; discussion 1275-7. 2006
    Unfavorable cardiac mechanics in children with hypoplastic left heart syndrome (HLHS) when compared with other single-ventricle defects may affect long-term morbidity and outcome...
  44. ncbi Aortic morphometry and microcephaly in hypoplastic left heart syndrome
    Amanda J Shillingford
    Division of Cardiology, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Cardiol Young 17:189-95. 2007
    ..Patients with hypoplastic left heart syndrome have been found to have an increased incidence of abnormal neurodevelopmental outcomes...
  45. ncbi Growth and correlates of nutritional status among infants with hypoplastic left heart syndrome (HLHS) after stage 1 Norwood procedure
    Deanne K Kelleher
    Division of GI Nutrition, Children s Hospital Boston, Boston, Massachusetts, USA
    Nutrition 22:237-44. 2006
    Protein-energy malnutrition is common among infants with congenital heart disease. We hypothesized that infants with hypoplastic left heart syndrome (HLHS) are at risk for malnutrition.
  46. ncbi Hypoplastic left heart syndrome: from in-utero diagnosis to school age
    Jack Rychik
    Fetal Heart Program, The Cardiac Center at The Children s Hospital of Philadelphia, and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Semin Fetal Neonatal Med 10:553-66. 2005
    ..New therapies and treatments will certainly arise to meet the challenges these children face as they enter into adulthood, and as our understanding of this unique cardiovascular state progresses...
  47. ncbi Anesthetic management of infants with palliated hypoplastic left heart syndrome undergoing laparoscopic nissen fundoplication
    Edward R Mariano
    Department of Anesthesia, University of CA at San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103 8770, USA
    Anesth Analg 100:1631-3. 2005
    ..We present the anesthetic management of five infants with palliated hypoplastic left heart syndrome that underwent laparoscopic Nissen fundoplication...
  48. ncbi Ventricular function deteriorates with recurrent coarctation in hypoplastic left heart syndrome
    Luis Alesandro Larrazabal
    Department of Cardiac Surgery, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Ann Thorac Surg 86:869-74; discussion 869-74. 2008
    Recurrent coarctation (re-CoA) after stage I palliation in hypoplastic left heart syndrome (HLHS) is deleterious. We studied whether re-CoA had an effect on ventricular systolic function.
  49. ncbi Right ventricular aneurysm following modified Norwood-Sano operation for hypoplastic left heart syndrome
    Mohsen Karimi
    Rainbow Heart Center, Department of Pediatric Cardiothoracic Surgery, Rainbow Babies and Children s Hospital, Case Western Reserve School of Medicine, 11100 Euclid Avenue, Cleveland, OH, USA
    Interact Cardiovasc Thorac Surg 7:664-6. 2008
    b>Hypoplastic left heart syndrome is a rare congenital heart defect characterized by underdevelopment of left-sided heart structures, including the aortic arch...
  50. ncbi Management of infants With hypoplastic left heart syndrome integrating research in Nursing practice
    Deborah Soetenga
    Pediatric Intensive Care Unit, Cardiovascular Surgery Program at Children s Hospital of Wisconsin, Milwaukee, Wis, USA
    Crit Care Nurse 24:46-8, 50, 52 passim. 2004
  51. ncbi Decreasing number of deaths of infants with hypoplastic left heart syndrome
    Brent M Gordon
    Division of Pediatric Cardiology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
    J Pediatr 153:354-8. 2008
    To assess mortality rates and demographic characteristics for infants with hypoplastic left heart syndrome (HLHS) in California.
  52. pmc Linkage analysis of left ventricular outflow tract malformations (aortic valve stenosis, coarctation of the aorta, and hypoplastic left heart syndrome)
    Kim L McBride
    Center for Molecular and Human Genetics, The Research Institute at Nationwide Children s Hospital, Columbus, OH 43205, USA
    Eur J Hum Genet 17:811-9. 2009
    ..outflow tract (LVOT) malformations aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS) are significant causes of infant mortality...
  53. ncbi Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome
    Jeffrey H Shuhaiber
    Loyola University Stritch School of Medicine, Chicago 60612, USA
    J Thorac Cardiovasc Surg 131:478-9. 2006
  54. ncbi Right ventricular performance in the fetus with hypoplastic left heart syndrome
    Anita Szwast
    The Fetal Heart Program, Cardiac Center, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Ann Thorac Surg 87:1214-9. 2009
    In the fetus with hypoplastic left heart syndrome (HLHS), the single right ventricle (RV) pumps the entire cardiac output in utero...
  55. ncbi Effect of shunt type on echocardiographic indices after initial palliations for hypoplastic left heart syndrome: Blalock-Taussig shunt versus right ventricle-pulmonary artery conduit
    Peter C Frommelt
    Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
    J Am Soc Echocardiogr 20:1364-73. 2007
    The study objective was to compare echocardiographic indices in infants with hypoplastic left heart syndrome (HLHS) randomized to different surgical pathways.
  56. ncbi Antenatal MR imaging of pulmonary lymphangiectasia secondary to hypoplastic left heart syndrome
    Mike Seed
    Department of Paediatric Cardiology, Hospital for Sick Children, Toronto, ON, Canada
    Pediatr Radiol 39:747-9. 2009
    We report a case of pulmonary lymphangiectasia detected antenatally with MRI in a fetus with hypoplastic left heart syndrome and a restrictive atrial septum.
  57. ncbi Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome
    Mio Taketazu
    Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    J Am Coll Cardiol 43:1902-7. 2004
    ..whether direct foramen ovale (FO) assessment or pulmonary venous (PV) flow patterns in fetal hypoplastic left heart syndrome (HLHS) correlate with clinical markers of postnatal left atrial (LA) hypertension severity associated ..
  58. pmc Center variation and outcomes associated with delayed sternal closure after stage 1 palliation for hypoplastic left heart syndrome
    Jason N Johnson
    Division of Pediatric Cardiology, Department of Pediatrics, Duke University Medical Center, Durham, NC 27715, USA
    J Thorac Cardiovasc Surg 139:1205-10. 2010
    ..debate whether primary or delayed sternal closure is the best strategy after stage 1 palliation for hypoplastic left heart syndrome. We describe center variation in delayed sternal closure after stage 1 palliation and associated ..
  59. pmc Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial
    Jane W Newburger
    Children s Hospital Boston and Harvard Medical School, MA, USA
    Circulation 125:2081-91. 2012
    ..Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass...
  60. ncbi Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome
    Meryl S Cohen
    Department of Pediatrics, University of Pennsylvania School of Medicine, Cardiac Center at Children s Hospital of Philadelphia, 19104, USA
    J Am Coll Cardiol 42:533-40. 2003
    We sought to assess the prevalence and progression of neo-aortic root dilation and valvar regurgitation after staged reconstruction for hypoplastic left heart syndrome (HLHS).
  61. ncbi Novel insights into RV adaptation and function in hypoplastic left heart syndrome between the first 2 stages of surgical palliation
    Nee Scze Khoo
    University of Alberta, Stollery Children s Hospital, Edmonton, Alberta, Canada
    JACC Cardiovasc Imaging 4:128-37. 2011
    This study sought to examine the changes in ventricular function of hypoplastic left heart syndrome (HLHS) between the first 2 stages of surgical palliation.
  62. ncbi Parental decision making regarding treatment of hypoplastic left heart syndrome
    Marcia K Ellinger
    Alberta Health Services, Northern Alberta Neonatal Intensive Care Program, Edmonton, Alberta, Canada
    Adv Neonatal Care 10:316-22; quiz 323-4. 2010
    Parents of children diagnosed with a complex congenital heart anomaly such as hypoplastic left heart syndrome (HLHS) are faced with the challenge of choosing the best treatment option for their children...
  63. ncbi Bilateral sensory permanent hearing loss after palliative hypoplastic left heart syndrome operation
    Charlene M T Robertson
    Department of Pediatrics, University of Alberta and Stollery Children s Hospital, Edmonton, Canada
    Ann Thorac Surg 93:1248-53. 2012
    ..report the prevalence and severity of PHL after Norwood right ventricular-pulmonary artery shunt for hypoplastic left heart syndrome (HLHS), document progressive loss, and explore markers of acute illness and ototoxic medications for ..
  64. ncbi Disturbed myocardial connexin 43 and N-cadherin expressions in hypoplastic left heart syndrome and borderline left ventricle
    Edris A F Mahtab
    Department of Anatomy and Embryology, Leiden University Medical Center LUMC, Leiden, The Netherlands
    J Thorac Cardiovasc Surg 144:1315-22. 2012
    Borderline left ventricle is the left ventricular morphology at the favorable end of the hypoplastic left heart syndrome. In contrast to the severe end, it is suitable for biventricular repair...
  65. ncbi Noncardiac surgery in children with hypoplastic left heart syndrome
    Adalberto Torres
    Division of Critical Care, Department of Pediatrics, University of Illinois College of Medicine and Children s Hospital of Illinois at St Francis Medical Center, Peoria, IL 61637, USA
    J Pediatr Surg 37:1399-403. 2002
    Hospital mortality rate among children with hypoplastic left heart syndrome (HLHS) after cardiac repair is well documented, but comparable data after noncardiac, surgical procedures are unknown...
  66. ncbi Parenting children with hypoplastic left heart syndrome: finding a balance
    Anra Lee
    Alberta Children s Hospital, Calgary, Canada
    J Spec Pediatr Nurs 16:179-89. 2011
    ..This study described the role normalization played for parents related to their young children with hypoplastic left heart syndrome. DESIGN AND METHODS. The data source for this secondary analysis was 30 interviews with 16 parents...
  67. ncbi Ethical principles and parental choice: treatment options for neonates with hypoplastic left heart syndrome
    Vicki L Zeigler
    College of Nursing, and Center for Nonlinear Science, Texas Woman s University, Denton, TX, USA
    Pediatr Nurs 29:65-9. 2003
    ..congenital heart disease face unique challenges, especially when caring for neonates diagnosed with hypoplastic left heart syndrome (HLHS)...
  68. ncbi Submicroscopic chromosomal copy number variations identified in children with hypoplastic left heart syndrome
    Ashleigh R Payne
    Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Pediatr Cardiol 33:757-63. 2012
    b>Hypoplastic left heart syndrome (HLHS), one of the most severe types of congenital heart disease (CHD), results in significant morbidity and mortality despite surgical palliation...
  69. ncbi Congenital Heart Surgery Nomenclature and Database Project: hypoplastic left heart syndrome
    C I Tchervenkov
    Division of Cardiovascular Surgery, The Montreal Children s Hospital, McGill University, Quebec, Canada
    Ann Thorac Surg 69:S170-9. 2000
    b>Hypoplastic left heart syndrome (HLHS) encompasses a spectrum of structural cardiac malformations that are characterized by severe underdevelopment of the structures in the left heart-aorta complex, including the left ventricular cavity ..
  70. ncbi Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking
    Colin Petko
    Klinik für angeborene Herzfehler und Kinderkardiologie, Universitatsklinikum Schleswig Holstein, Kiel, Germany
    Cardiol Young 21:677-83. 2011
    The left ventricle in patients with hypoplastic left heart syndrome may influence right ventricular function and outcome...
  71. ncbi The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome
    J N Graziano
    Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, MI 48109, USA
    Pediatr Cardiol 23:146-51. 2002
    b>Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality...
  72. ncbi Balloon angioplasty for recurrent coarctation of the aorta in patients following staged palliation for hypoplastic left heart syndrome
    T M Zellers
    Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas, 75235, USA
    Am J Cardiol 84:231-3, A9. 1999
    After stage I palliation for hypoplastic left heart syndrome, recurrent coarctation occurred in 9 of 25 survivors (36%) requiring balloon angioplasty, predominantly from a prograde approach...
  73. ncbi Evolving strategies and improving outcomes of the modified norwood procedure: a 10-year single-institution experience
    T Azakie
    Department of Surgery, The Hospital for Sick Children, University of Toronto School of Medicine, Ontario, Canada
    Ann Thorac Surg 72:1349-53. 2001
    ..This study reviews our 10-year experience with the modified Norwood procedure to determine its early and midterm outcomes. The focus is on the impact of evolving management strategies and accumulated institutional experience...
  74. ncbi Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center
    Vladimiro L Vida
    Department of Cardiac Surgery, Children s Hospital Boston, Boston, Massachusetts 02115, USA
    Ann Thorac Surg 84:581-5; discussion 586. 2007
    ..atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study...
  75. ncbi Cardiac magnetic resonance imaging prior to bidirectional cavopulmonary connection in hypoplastic left heart syndrome
    Bryn O Jones
    Department of Cardiology, The Royal Children s Hospital, Parkville, Melbourne, Australia
    Heart Lung Circ 19:535-40. 2010
    ..obtained from cardiac catheterisation is not essential in pre-operative assessment of children with hypoplastic left heart syndrome (HLHS) undergoing Bidirectional Cavopulmonary Connection (BCPC)...
  76. ncbi Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience
    J Rychik
    Fetal Heart Program, The Cardiac Center, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Ultrasound Obstet Gynecol 36:465-70. 2010
    To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS)...
  77. ncbi First-stage palliation for hypoplastic left heart syndrome in the twenty-first century
    Jeffrey M Pearl
    Division of Pediatric Cardiothoracic Surgery, Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Ann Thorac Surg 73:331-9; discussion 339-40. 2002
    ..physiology and experience with the surgical techniques and perioperative care of patients with hypoplastic left heart syndrome have resulted in improved outcomes...
  78. ncbi Total cavopulmonary connections in children with a previous Norwood procedure
    A Azakie
    Department of Surgery, The Hospital for Sick Children, University of Toronto School of Medicine, Ontario, Canada
    Ann Thorac Surg 71:1541-6. 2001
    ..6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation...
  79. ncbi Comprehensive evaluation of right ventricular function in children with different anatomical subtypes of hypoplastic left heart syndrome after Fontan surgery
    Colin Petko
    Children s Heart Centre, University Hospital of Schleswig Holstein, Campus Kiel, Schwanenweg 20, 24105 Kiel, Germany
    Int J Cardiol 150:45-9. 2011
    There is some evidence that hypoplastic left heart syndrome (HLHS) survivors with a larger left ventricular (LV) cavity may have poorer long-term outcome than those with mitral and aortic valve atresia (MA/AA) and negligible LV...
  80. ncbi The hypoplastic left heart syndrome is not a risk factor for Fontan operation
    Artur Henrique de Souza
    Rev Bras Cir Cardiovasc 25:506-9. 2010
    To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality.
  81. ncbi Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome
    M Galantowicz
    The Heart Center, Columbus Children s Hospital, Columbus, OH 43205, USA
    Pediatr Cardiol 26:190-9. 2005
    ..techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal...
  82. ncbi Early postoperative outcomes in a series of infants with hypoplastic left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle to pulmonary artery conduit
    Clifford L Cua
    Department of Cardiology, Children s Hospital, Boston, MA, USA
    Pediatr Crit Care Med 7:238-44. 2006
    ..publications using nonconcurrent series of patients indicate improved survival for patients with hypoplastic left heart syndrome (HLHS) undergoing stage I palliation with a right ventricle to pulmonary artery conduit (NW-RVPA) vs...
  83. ncbi Right ventricle to pulmonary artery conduit reduces interim mortality after stage 1 Norwood for hypoplastic left heart syndrome
    Christian Pizarro
    Nemours Cardiac Center, Alfred I duPont Hospital for Children, Wilmington, Delaware, USA
    Ann Thorac Surg 78:1959-63; discussion 1963-4. 2004
    ....
  84. ncbi Is "treat your child normally" helpful advice for parents of survivors of treatment of hypoplastic left heart syndrome?
    Gwen R Rempel
    Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada
    Cardiol Young 19:135-44. 2009
    ..Little is known about how mothers and fathers experience parenting a child whose new state as a survivor may include less than optimal developmental sequels...
  85. pmc Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome
    Doff B McElhinney
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    Circulation 120:1482-90. 2009
    ..in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings...
  86. pmc Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart
    Sitaram M Emani
    Children s Hospital Boston, Boston, MA 02115, USA
    J Thorac Cardiovasc Surg 138:1276-82. 2009
    ..We studied the effects of left ventricular rehabilitation--endocardial fibroelastosis resection with mitral or aortic valvuloplasty--on left ventricular function and clinical outcomes...
  87. ncbi Morphometry of coronary capillaries in hypoplastic left heart syndrome
    Caner Salih
    Departments of Pediatrics and Histopathology, Imperial College, London, United Kingdom
    Ann Thorac Surg 77:903-7; discussion 907. 2004
    b>Hypoplastic left heart syndrome is a condition characterized by a constellation of morphological malformations affecting the left side of the heart...
  88. ncbi Neurodevelopment in children with hypoplastic left heart syndrome
    Anne Sarajuuri
    Division of Child Neurology, Department of Gynecology and Pediatrics, Helsinki University Central Hospital, Helsinki, Finland
    J Pediatr 157:414-20, 420.e1-4. 2010
    To assess neurodevelopment in children with hypoplastic left heart syndrome (HLHS) or other types of functionally single ventricle (univentricular heart, UVH) and to estimate the effect of possible clinical predictors on outcome.
  89. ncbi Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS)
    Aymen N Naguib
    Department of Anesthesia The Heart Center, Nationwide Children s Hospital, Columbus, OH 43205, USA
    Paediatr Anaesth 20:38-46. 2010
    Despite advances in the surgical and perioperative management of patients with hypoplastic left heart syndrome (HLHS), outcomes for this high-risk group of patients remains suboptimal...
  90. ncbi Hybrid approach as bridge to biventricular repair in a neonate with critical aortic stenosis and borderline left ventricle
    Stephen C Brown
    Pediatric Cardiology, University of the Free State, Bloemfontein, South Africa
    Eur J Cardiothorac Surg 35:1080-2. 2009
    ..The hybrid approach allowed adequate time (months) for careful consideration and acted as a bridge to biventricular repair in this infant...
  91. ncbi Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome: patient selection for fetal intervention
    Kaarin Mäkikallio
    Department of Cardiology, Children s Hospital, Harvard Medical School, Boston, MA, USA
    Circulation 113:1401-5. 2006
    Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention...
  92. ncbi Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy
    Emile A Bacha
    Department of Congenital and Pediatric Cardiac Surgery, The University of Chicago Children s Hospital, Chicago, Ill, USA
    J Thorac Cardiovasc Surg 131:163-171.e2. 2006
    Survival after stage I palliation for hypoplastic left heart syndrome or related anomalies remains poor in high-risk neonates. We hypothesized that a less invasive hybrid approach would be beneficial in this patient population.
  93. ncbi Quality of life in children with hypoplastic left heart syndrome
    M Mellander
    Department of Pediatric Cardiology, The Queen Silvia Children s Hospital, Goteborg, University, Goteborg, Sweden
    Acta Paediatr 96:53-7. 2007
    To measure quality of life in children with hypoplastic left heart syndrome and their families.
  94. ncbi Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome
    Jeffrey Phillip Jacobs
    The Congenital Heart Institute of Florida, All Children s Hospital, University of South Florida, Saint Petersburg, FL 33701, USA
    Cardiol Young 16:556-62. 2006
    Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients.
  95. ncbi Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart
    Hakan Akintuerk
    Pediatric Heart Center, Justus Liebig University Giessen, Germany
    Circulation 105:1099-103. 2002
    ..But it remains to be elucidated whether this approach allows a new strategy for patients with HLH...
  96. ncbi Hypoplastic left heart syndrome: diagnosis and management
    Rachel Andrews
    Department of Congenital Heart Disease, Guy s Hospital, Guy s and St Thomas NHS Trust, London SE1 9RT
    Hosp Med 63:24-7. 2002
    The prognosis of hypoplastic left heart syndrome has improved dramatically in recent years with advances in reconstructive surgery...
  97. pmc Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis
    R Andrews
    Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas' NHS Trust, London, UK
    Arch Dis Child 85:474-7. 2001
    BACKGROUND AND AIMS: Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options...
  98. ncbi Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome
    William T Mahle
    Children s Healthcare of Atlanta, Atlanta, GA 30322 1062, USA
    Pediatrics 117:e90-7. 2006
    Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure...
  99. ncbi Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome
    Keiji Haseyama
    Department of Pediatric Cardiology and Pediatric Cardiac Surgery, Nagano Children s Hospital, Nagano, Japan
    J Thorac Cardiovasc Surg 132:1232-3. 2006
  100. ncbi Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome
    W Tworetzky
    Divisions of Pediatric Cardiology and Cardiothoracic Surgery, University of California, San Francisco, USA
    Circulation 103:1269-73. 2001
    b>Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.
  101. ncbi Mixed venous oxygen saturation monitoring after stage 1 palliation for hypoplastic left heart syndrome
    James S Tweddell
    Herma Heart Center and Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, Wisconsin 53226, USA
    Ann Thorac Surg 84:1301-10; discussion 1310-1. 2007
    Staged palliation for hypoplastic left heart syndrome has been marked by high early mortality due to the limited cardiac output of the postischemic single right ventricle combined with the inefficiency and volatility of parallel ..

Research Grants33

  1. THE GENETIC ETIOLOGY OF HYPOPLASTIC LEFT HEART SYNDROME
    Peter Bowers; Fiscal Year: 2004
    b>Hypoplastic left heart syndrome (HLHS) is one of the most lethal forms of congenital cardiovascular malformation...
  2. MOLECULAR MECHANISMS OF DIOXIN ACTION
    Alvaro Puga; Fiscal Year: 2013
    ..Valvular stenosis and hypoplastic left heart syndrome are the most common malformations occurring in eight out of 1000 live births and constituting 25-30% ..
  3. Hypoplastic Left Heart Syndrome: Expression of RHD in the Fetus?
    Pirooz Eghtesady; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Hypoplastic Left Heart Syndrome (HLHS) is a severe and devastating heart defect that affects ~ 1 in 10,000 children born each year...
  4. The Role of Histone Deacytelases in Right Ventricular Adaptation in Hypoplastic L
    SHELLEY DEANNE MIYAMOTO; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Hypoplastic Left Heart Syndrome (HLHS) is the most common form of severe congenital heart disease and is the leading cause of cardiovascular death in infancy...
  5. Pathogenetics of the FOX transcription factor gene cluster on 16q24.1
    Pawel Stankiewicz; Fiscal Year: 2013
    ..Pleiotropic effects encountered in FOXF1 microdeletions, such as hypoplastic left heart syndrome and gastrointestinal atresias, may be due to haploinsufficiency for the neighboring genes, FOXC2 and ..
  6. Identification of a Stretch-Activated Channel with a Role in Cardiac Development
    Dipayan Chaudhuri; Fiscal Year: 2013
    ..of flow can produce congenital defects, such as the severe cardiomyocyte hypo- proliferation seen in hypoplastic left heart syndrome. Yet, the identity of key mechanosensors remains unknown...
  7. Exome Sequencing in Familial Cardiovascular Malformations
    KIM LEWIS MCBRIDE; Fiscal Year: 2012
    ..outflow tract (LVOT) malformations that includes aortic valve stenosis, coarctation of the aorta, and hypoplastic left heart syndrome. There is a large genetic component to these malformations, but few susceptibility genes have been ..
  8. Pediatric Heart Network University of Utah
    RICHARD VINCENT WILLIAMS; Fiscal Year: 2013
    ..This study addresses the significant problem of growth failure in infants with hypoplastic left heart syndrome (HLHS)...
  9. Neurodevelopmental Outcomes in Infants with HLHS after Hybrid Stage I Palliation
    Sharon L Hill; Fiscal Year: 2011
    DESCRIPTION (provided by applicant): Hypoplastic Left Heart Syndrome remains the leading cause of death of all congenital heart defects in the first year of life...
  10. Preclinical Evaluation of the Adjustable Systemic-Pulmonary Artery Shunt
    MOHAMMAD WAQAR MOHIUDDIN; Fiscal Year: 2012
    ..surgery for neonatal single ventricle birth defect, particularly after the Norwood procedure for hypoplastic left heart syndrome. Contributing to the high mortality rates is the inability to precisely control pulmonary blood flow ..
  11. Pediatric Heart Network Regents of the University of Michigan
    Richard G Ohye; Fiscal Year: 2013
    ..heart disease (CHD) lesions is the Functional Single Right Ventricle (FSRV) malformations, including hypoplastic left heart syndrome. Survival beyond early infancy, once impossible for this patient group, has improved markedly in the ..
  12. Developing an animal model of HLHS: role of immune mediated injury
    Pirooz Eghtesady; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Hypoplastic left heart syndrome (HLHS) is a severe and devastating heart defect that affects ~ 1 in 5-10,000 children born each year and accounts for 25% of all neonatal deaths from congenital heart ..
  13. Excitation-contraction coupling in human ventricle development
    Mary B Wagner; Fiscal Year: 2012
    ..compare properties of biopsies removed at less than one week of age (newborns) from children with hypoplastic left heart syndrome to ventricular biopsies removed at 3-12 months (infants) of age from children with Tetralogy of ..
  14. Pediatric Heart Network: Follow-up of the Single Ventricle Reconstruction Trial
    Wyman Lai; Fiscal Year: 2010
    ..Relevance to public health: Surgery for hypoplastic left heart syndrome has the highest incidence of death or transplantation of all congenital heart disease lesions...
  15. Towards repairing congenital heart defects: The effects of fetal cardiac extracel
    Corin Williams; Fiscal Year: 2013
    ..b>Hypoplastic Left Heart Syndrome (HLHS) is a rare CHD that requires several major reconstructive surgeries (3)...
  16. Genetic and Developmental Basis of Pediatric Aortic Valve Disease Pathogenesis
    ROBERT BRUCE HINTON; Fiscal Year: 2010
    ..Using families identified by a hypoplastic left heart syndrome (HLHS) proband, parametric and nonparametric linkage analysis will be performed to identify aortic ..
  17. MOLECULAR BASIS FOR HYPOPLASTIC HEART SYNDROMES
    Eric Olson; Fiscal Year: 2003
    ..in these cardiac compartments in three human syndromes associated with congenital heart disease: hypoplastic left heart syndrome, Down syndrome, and DiGeorge syndrome...
  18. Bridge to neonatal Fontan repair of single ventricle
    Mark Rodefeld; Fiscal Year: 2006
    ..The most common form, hypoplastic left heart syndrome, in which the left ventricle fails to form in a way that is ever functional, is the leading cause of ..
  19. Computer Assisted Surgery Prenatal Cardiac Intervention
    Constantinos Nikou; Fiscal Year: 2004
    ..is highly desirable to correct aortic valve stenoses and other abnormalities that lead to hypoplastic left heart syndrome (HLHS). At the same time the technology available for PCI is clearly deficient...
  20. IMPROVING OUTCOMES IN HYPOPLASTIC LEFT HEART SYNDROME
    Pamela Jenkins; Fiscal Year: 2004
    ..Her current proposal concentrates on the health outcomes of infants with hypoplastic left heart syndrome (HLHS), a cardiovascular malformation whose surgical treatments still have a high mortality...
  21. High-resolution genome scans in congenital heart disease
    Dorothy Warburton; Fiscal Year: 2009
    ..genome scanning for copy number changes (CNCs), to search for such lesions in 115 patients with hypoplastic left heart syndrome and 260 patients with conotruncal heart disease...
  22. FAMILY STUDY OF CONGENITAL CARDIOVASCULAR MALFORMATIONS
    Joann Boughman; Fiscal Year: 1992
    ..To investigate the risks associated with left outflow tract defects, the families of children with hypoplastic left heart syndrome or coarctation of the aorta will be studied...
  23. The Role of IgLONS in Cardiac Development and Disease
    Paul Grossfeld; Fiscal Year: 2007
    ..for incorporating human and animal genetic model system approaches for studying genes causing hypoplastic left heart syndrome (HLHS). UCSD and specifically, Dr...
  24. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2007
    ..Left-sided cardiac defects (LSCD) include valvar aortic stenosis, coarctation of the aorta and hypoplastic left heart syndrome, and account for at least 10% of CHD...
  25. APOE Genotype and Sequelae of Infant Cardiac Surgery
    James Gaynor; Fiscal Year: 2007
    ..unreadable] [unreadable]..
  26. Mentored Patient-Oriented Research Career Development Award
    Ruey Kang Chang; Fiscal Year: 2006
    ..The principal investigator will take advanced courses to enhance knowledge and skills needed for patient-oriented research and work with the mentors to conduct the proposed project during the award period. ..
  27. Decisionmaking in Pediatric Palliative Care
    JOHN FEUDTNER; Fiscal Year: 2007
    ..unreadable] [unreadable] [unreadable]..
  28. High Dose Erythropoetin Neuroprotection Trial for Neonatal Cardiac Surgery
    Dean Andropoulos; Fiscal Year: 2008
    ..This study will show if babies that are given erythropoetin have fewer delays in development than babies that do not receive the drug. [unreadable] [unreadable] [unreadable]..
  29. Genetics of Congenital Left-sided Heart Defects
    Kim McBride; Fiscal Year: 2007
    ..The proposed studies will launch the applicant on an independent research career, providing a base on which to advance genetic analyses of CCVM with the aim to reduce their occurrence and provide new treatment opportunities. ..
  30. Prenatal Ultrasound And Birth Defects
    DOROTHY WALLER; Fiscal Year: 2008
    ..Estimates of sensitivity and specificity will be calculated for each of 38 different categories of malformations. [unreadable] [unreadable] [unreadable]..
  31. The Role of Eomesodermin in Cardiac Development
    Kenneth Ryan; Fiscal Year: 2007
    ..AIM 4. In situ hybridization is used to determine the full range of cardiac cell types expressing Eomes; a conditional dominant-negative isoform of Eomes examines the requirement for Eomes in the heart. ..
  32. Multidisciplinary Study of Right Ventricular Dysplasia
    Jeffrey Towbin; Fiscal Year: 2005
    ..This integrated research grant proposal offers a substantial prospect of expanding the fund of clinical knowledge regarding ARVD and of localizing the gene(s) responsible for this disorder. ..
  33. VOLATILE ANESTHETICS AND RESPIRATORY NEUROTRANSMISSION
    Eckehard Stuth; Fiscal Year: 2009
    ....