Robert B Hinton

Summary

Affiliation: Cincinnati Children's Hospital Medical Center
Country: USA

Publications

  1. pmc Bicuspid aortic valve and thoracic aortic aneurysm: three patient populations, two disease phenotypes, and one shared genotype
    Robert B Hinton
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, 240 Albert Sabin Way, Cincinnati, OH 45219, USA
    Cardiol Res Pract 2012:926975. 2012
  2. pmc Elastin haploinsufficiency results in progressive aortic valve malformation and latent valve disease in a mouse model
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Circ Res 107:549-57. 2010
  3. pmc A fetus with hypertrophic cardiomyopathy, restrictive, and single-ventricle physiology, and a beta-myosin heavy chain mutation
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Pediatr 157:164-6. 2010
  4. 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
  5. pmc The family history: reemergence of an established tool
    Robert B Hinton
    Division of Cardiology, MLC 2003, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Crit Care Nurs Clin North Am 20:149-58, v. 2008
  6. ncbi request reprint Mouse heart valve structure and function: echocardiographic and morphometric analyses from the fetus through the aged adult
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Heart Circ Physiol 294:H2480-8. 2008
  7. pmc Maladaptive matrix remodeling and regional biomechanical dysfunction in a mouse model of aortic valve disease
    Varun K Krishnamurthy
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Matrix Biol 31:197-205. 2012
  8. pmc Differential expression of cartilage and bone-related proteins in pediatric and adult diseased aortic valves
    Elaine E Wirrig
    Division of Molecular Cardiovascular Biology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Mol Cell Cardiol 50:561-9. 2011
  9. 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
  10. ncbi request reprint 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

Collaborators

Detail Information

Publications31

  1. pmc Bicuspid aortic valve and thoracic aortic aneurysm: three patient populations, two disease phenotypes, and one shared genotype
    Robert B Hinton
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, 240 Albert Sabin Way, Cincinnati, OH 45219, USA
    Cardiol Res Pract 2012:926975. 2012
    ....
  2. pmc Elastin haploinsufficiency results in progressive aortic valve malformation and latent valve disease in a mouse model
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Circ Res 107:549-57. 2010
    ..Because elastic fiber abnormalities are a central feature of degenerative valve disease, we hypothesized that elastin-insufficient mice would manifest viable heart valve disease...
  3. pmc A fetus with hypertrophic cardiomyopathy, restrictive, and single-ventricle physiology, and a beta-myosin heavy chain mutation
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Pediatr 157:164-6. 2010
    ..We describe a patient with a pathogenic familial beta-myosin heavy chain mutation who was prenatally diagnosed with left ventricular hypoplasia and restrictive diastolic physiology...
  4. 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
    ..Brains from HLHS fetuses demonstrated chronic diffuse white matter injury of varying severity. These patterns of prenatal head growth and brain histopathology identify a spectrum of abnormal CNS development and/or injury in HLHS fetuses...
  5. pmc The family history: reemergence of an established tool
    Robert B Hinton
    Division of Cardiology, MLC 2003, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Crit Care Nurs Clin North Am 20:149-58, v. 2008
    ..The increasing use of genetic screening promises to cultivate a paradigm shift in medical treatment emphasizing primary prevention and early intervention. Appreciation of the family history is necessary to make this important advance...
  6. ncbi request reprint Mouse heart valve structure and function: echocardiographic and morphometric analyses from the fetus through the aged adult
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
    Am J Physiol Heart Circ Physiol 294:H2480-8. 2008
    ....
  7. pmc Maladaptive matrix remodeling and regional biomechanical dysfunction in a mouse model of aortic valve disease
    Varun K Krishnamurthy
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Matrix Biol 31:197-205. 2012
    ..Combining molecular and engineering approaches provides complementary mechanistic insights that may be informative in the search for new therapeutic targets and durable valve bioprostheses...
  8. pmc Differential expression of cartilage and bone-related proteins in pediatric and adult diseased aortic valves
    Elaine E Wirrig
    Division of Molecular Cardiovascular Biology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    J Mol Cell Cardiol 50:561-9. 2011
    ..These findings provide specific molecular indicators of AoVD progression, which may lead to identification of early disease markers and the development of potential therapeutics...
  9. 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)...
  10. ncbi request reprint 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...
  11. ncbi request reprint Somatic growth trajectory in the fetus with hypoplastic left heart syndrome
    James F Cnota
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
    Pediatr Res 74:284-9. 2013
    ..Fetal growth trajectory has not been described. We hypothesized that fetal growth trajectory declines across late gestation in this population...
  12. pmc Twist1 promotes heart valve cell proliferation and extracellular matrix gene expression during development in vivo and is expressed in human diseased aortic valves
    Santanu Chakraborty
    The Heart Institute, Cincinnati Children s Medical Center, Cincinnati, OH 45229, USA
    Dev Biol 347:167-79. 2010
    ..Overall, these data implicate Twist1 as a critical regulator of valve development and suggest that Twist1 influences ECM production and cell proliferation during disease...
  13. pmc Pediatric cardiomyopathy: importance of genetic and metabolic evaluation
    Steven J Kindel
    Department of Pediatrics, Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    J Card Fail 18:396-403. 2012
    ..A research-based pediatric cardiomyopathy registry identified familial, syndromic, or metabolic causes in 30% of children. However, these results predated clinical genetic testing...
  14. ncbi request reprint Bilateral semilunar valve disease in a child with partial deletion of the Williams-Beuren syndrome region is associated with elastin haploinsufficiency
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital, Cincinnati, Ohio 45229 3039, USA
    J Heart Valve Dis 15:352-5. 2006
    ..Histochemical analysis of the aortic valve revealed decreased and disorganized elastin with loss of the normal trilaminar cusp organization. These findings suggest that elastin has a role in the pathogenesis of semilunar valve disease...
  15. pmc Regional structure-function relationships in mouse aortic valve tissue
    Varun K Krishnamurthy
    Department of Biomedical Engineering, University of Cincinnati, Cincinnati, OH, USA
    J Biomech 44:77-83. 2011
    ..The micropipette aspiration technique provides a promising approach for studies of valve structure and function in small animal models, such as transgenic mouse models of valve disease...
  16. ncbi request reprint A pediatric approach to family history of cardiovascular disease: diagnosis, risk assessment, and management
    Erin M Miller
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA Electronic address
    Pediatr Clin North Am 61:187-205. 2014
    ..Improved understanding of the causes of pediatric cardiovascular disease promises the opportunity to develop new diagnostic and therapeutic strategies. ..
  17. pmc Heart valve structure and function in development and disease
    Robert B Hinton
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Ohio 45229, USA
    Annu Rev Physiol 73:29-46. 2011
    ..Further studies are necessary to determine regulatory pathway interactions underlying valve pathogenesis in order to generate new avenues for novel therapeutics...
  18. ncbi request reprint Extracellular matrix remodeling and organization in developing and diseased aortic valves
    Robert B Hinton
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Circ Res 98:1431-8. 2006
    ....
  19. ncbi request reprint Evidence in favor of linkage to human chromosomal regions 18q, 5q and 13q for bicuspid aortic valve and associated cardiovascular malformations
    Lisa J Martin
    Center for Epidemiology and Biostatistics, University of Cincinnati, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
    Hum Genet 121:275-84. 2007
    ..These regions likely contain genes whose mutation results in BAV and/or associated CVM indicating their important role in valvulogenesis and cardiac development...
  20. ncbi request reprint Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome
    Patrick T Hangge
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
    Pediatr Res 74:61-7. 2013
    ..We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes...
  21. pmc TGF-β mediates early angiogenesis and latent fibrosis in an Emilin1-deficient mouse model of aortic valve disease
    Charu Munjal
    Division of Cardiology, The Heart Institute, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
    Dis Model Mech 7:987-96. 2014
    ..The early manifestation of EFF and aberrant angiogenesis suggests that these processes are crucial intermediate factors involved in disease progression and therefore might provide new therapeutic targets for human AVD. ..
  22. ncbi request reprint Replacement of the aortic valve in a patient with mucolipidosis III
    Linda H Cripe
    The Heart Institute, Cincinnati Children s Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, Ohio
    Cardiol Young 19:641-3. 2009
    ..As life expectancy increases for patients with lysosomal storage disorders, approaches to intervention for valvar disease become increasingly important...
  23. pmc The presence of bicuspid aortic valve does not predict ventricular septal defect type
    Kan N Hor
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Am J Med Genet A 146:3202-5. 2008
    ..This may be due to phenotypic and genetic heterogeneity of BAV and VSD, other modifying factors as manifested by differences in associated CVM, as well as limitations of the clinical taxonomy of VSD...
  24. doi request reprint Risk factors for aortic valve disease in bicuspid aortic valve: a family-based study
    Troy J Calloway
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Ohio 45229 3039, USA
    Am J Med Genet A 155:1015-20. 2011
    ..BAV is determined largely by genetic effects, but the phenotypic variability of AVD is primarily determined by nongenetic factors. BAV morphology may have predictive value for the time course of AVD...
  25. ncbi request reprint Genetic testing practices in infants with congenital heart disease
    Jessica A Connor
    Division of Human Genetics, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio, USA
    Congenit Heart Dis 9:158-67. 2014
    ..We hypothesized that chromosome microarray analysis (CMA) would identify genetic abnormalities underlying both syndromic and isolated CHD...
  26. ncbi request reprint Tetrasomy 15q25.2→qter identified with SNP microarray in a patient with multiple anomalies including complex cardiovascular malformation
    Jaya K George-Abraham
    Division of Human Genetics, Department of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Am J Med Genet A 158:1971-6. 2012
    ..Finally, we believe cardiac defects with this genetic syndrome are a poor prognostic finding associated with high mortality...
  27. ncbi request reprint Novel fibrillin 1 mutation in a case of neonatal Marfan syndrome: the increasing importance of early recognition
    Jamie Sutherell
    Division of Cardiology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
    Congenit Heart Dis 2:342-6. 2007
    ..Because of potential new therapies, it is increasingly important to recognize neonatal MFS in utero as well as shortly after birth to initiate the appropriate diagnostic work-up and management...
  28. ncbi request reprint BMP and FGF regulatory pathways in semilunar valve precursor cells
    Bin Zhao
    Division of Cardiology, MLC 7042, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229, USA
    Dev Dyn 236:971-80. 2007
    ....
  29. doi request reprint Hormonal regulation of prostaglandin E2 receptors: localization and expression in rat cervical tissue
    Andrea C Hinton
    Division of Maternal Fetal Medicine, Good Samaritan Hospital, Cincinnati, Ohio 45220, USA
    Reprod Sci 17:136-46. 2010
    ..Progesterone differentially regulates the expression of PGE2 receptor isoforms in the cervix. Elucidating the regulation of PGE2 receptors may facilitate improved approaches to the prevention and treatment of preterm labor...
  30. pmc Scleraxis is required for cell lineage differentiation and extracellular matrix remodeling during murine heart valve formation in vivo
    Agata K Levay
    Department of Molecular and Cellular Pharmacology, Leonard M Miller School of Medicine, University of Miami, Miami, FL 33101, USA
    Circ Res 103:948-56. 2008
    ..Collectively, our studies have identified an in vivo requirement for scx during valvulogenesis and demonstrate its role in cell lineage differentiation and matrix distribution in remodeling valve structures...

Research Grants2

  1. Genetic and Developmental Basis of Pediatric Aortic Valve Disease Pathogenesis
    Robert Hinton; Fiscal Year: 2007
    ..A Career Development Award will allow the Candidate to devote 80% effort to patient-oriented research, and to obtain the training necessary to transition to an independent investigator. (End of Abstract) ..
  2. Genetic and Developmental Basis of Pediatric Aortic Valve Disease Pathogenesis
    Robert Hinton; Fiscal Year: 2008
    ..A Career Development Award will allow the Candidate to devote 80% effort to patient-oriented research, and to obtain the training necessary to transition to an independent investigator. (End of Abstract) ..