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Title of Journal: Pediatr Cardiol

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Abbravation: Pediatric Cardiology

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Springer-Verlag

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DOI

10.1007/978-1-60327-459-3_14

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1432-1971

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Maternally Inherited Hypertrophic Cardiomyopathy

Authors: AI Dipchand I Tein B Robinson LN Benson
Publish Date: 2014/04/30
Volume: 22, Issue: 1, Pages: 14-22
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Abstract

In the past decade maternally inherited disorders have been described manifesting as hypertrophic cardiomyopathy These are primarily associated with defects in oxidative metabolism due to an alteration in mitochondrial DNA Although the biochemistry and molecular biology is welldefined there is little information regarding clinical presentation and course Reported manifestations can be broad and can include myopathy encephalopathy strokelike episodes hearing loss cardiomyopathy multiorgan dysfunction and sudden death Predominant or exclusive involvement of the heart is rare We report the clinical presentations investigations pathologic findings and clinical course in two families with two mitochondrial tRNA defects with exclusive cardiac involvement and demonstrable clinical heterogeneity based on the percentage of mutant tRNA

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Other Papers In This Journal:
  1. Incidence of Stenotic Lesions Predicted by Acute Phase Changes in Coronary Arterial Diameter During Kawasaki Disease
  2. Transcatheter Amplatzer Occlusion and Surgical Closure of Patent Ductus Arteriosus: Comparison of Effectiveness and Costs in a Low-Income Country
  3. Assessments of Coronary Artery Visibility and Radiation Dose in Infants with Congenital Heart Disease on Cardiac 128-slice CT and on Cardiac 64-slice CT
  4. From Other Journals
  5. Maternal Smoking During Pregnancy and the Risk of Congenital Heart Defects in Offspring: A Systematic Review and Metaanalysis
  6. Semi-supine Exercise Stress Echocardiography in Children and Adolescents: Feasibility and Safety
  7. Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy, Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus
  8. Medication Dosing and Renal Insufficiency in a Pediatric Cardiac Intensive Care Unit: Impact of Pharmacist Consultation
  9. Time Course of Myocardial Amiodarone Uptake in the Piglet Heart Using a Chronic Animal Model
  10. Time Course of Myocardial Amiodarone Uptake in the Piglet Heart Using a Chronic Animal Model
  11. Balloon Valvuloplasty for Critical Pulmonary Valve Stenosis in a Premature Infant
  12. “Baby Heart Project”: The Italian Project for Accreditation and Quality Management in Pediatric Cardiology and Cardiac Surgery
  13. Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without
  14. Does the Stage 16 Embryo in Hamburger–Hamilton’s “Series of Normal Stages in the Development of the Chick Embryo” Have a Potential “Conotruncal” Heart Defect?
  15. Beraprost Therapy in Children with Pulmonary Hypertension Secondary to Congenital Heart Disease
  16. Does the Stage 16 Embryo in Hamburger–Hamilton’s “Series of Normal Stages in the Development of the Chick Embryo” Have a Potential “Conotruncal” Heart Defect?
  17. Tako-Tsubo Syndrome in a 12-Year-Old Girl: Exhausted Heart, Not Broken Heart
  18. Administration of Steroids in Pediatric Cardiac Surgery: Impact on Clinical Outcome and Systemic Inflammatory Response
  19. Troponin I Elevation After Supraventricular Tachycardia in a Child with Hypertrophic Cardiomyopathy
  20. Interventions in Kawasaki Disease
  21. Pericardial Effusion in a Preterm Infant Resulting from Umbilical Venous Catheter Placement
  22. Clinical Improvement After Banding of a Pulmonary Branch Artery in a Symptomatic Patient with Osler–Rendu–Weber Syndrome
  23. Coarctation of the Aorta, Aortic Valvar Stenosis, and Supravalvar Aortic Stenosis with Left Coronary Artery Ostial Stenosis: Management Using a Staged Hybrid Approach
  24. Intrauterine Beta Blockade
  25. Food and Drug Administration (FDA) Postmarket Reported Side Effects and Adverse Events Associated with Pulmonary Hypertension Therapy in Pediatric Patients
  26. Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease
  27. Multicenter Study of the Effectiveness of Implantable Cardioverter Defibrillators in Children and Young Adults With Heart Disease
  28. Serum Alkaline Phosphatase Reflects Post-Fontan Hemodynamics in Children
  29. Pediatric Cardiology: Pediatrics or Cardiology?
  30. From Other Journals
  31. Pulmonary Atresia with Intact Ventricular Septum Coexisting with Coarctation of the Aorta
  32. Initial Experience with the Nit-Occlud ASD-R: Short-Term Results
  33. Echocardiographic Measurements of Cardiac Dimensions Correlate Better With Body Length Than With Body Weight or Body Surface Area
  34. Quantitative Ultrasound Measurement of Intima–Media Thickness of Abdominal Aorta and Common Carotid Arteries in Normal Term Newborns
  35. Abnormal Circumferential Strain is Present in Young Duchenne Muscular Dystrophy Patients
  36. Juxtaposition of the Atrial Appendages: A Clinical Series of 22 Patients
  37. Association of A Dilated Coronary Sinus in the Fetus with Actual and Apparent Coarctation of the Aorta and Diminutive Left Heart Structures
  38. Increased Incidence of Incomplete Kawasaki Disease at a Pediatric Hospital After Publication of the 2004 American Heart Association Guidelines
  39. Plastic bronchitis in children with fontan palliation: Analogue to protein losing enteropathy?
  40. Screening Newborns for Congenital Heart Disease with Pulse Oximetry: Survey of Pediatric Cardiologists
  41. Late Ventricular Potentials and QT Dispersion in Familial Dysautonomia
  42. Combined Treatment for Multiple Cardiac Defects with Interventional Techniques
  43. The Impact of Gestational Age on Resource Utilization After Open Heart Surgery for Congenital Cardiac Disease From Birth to 1 Year of Age
  44. Neurophysiological Effects of Pediatric Balloon Dilatation Procedures
  45. Inhaled Nitric Oxide Therapy in a Critically Ill Neonate with Ebstein's Anomaly
  46. Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery
  47. Prenatal Detection of Coarctation of the Aorta in a Non-selected Population: A Prospective Analysis of 10 Years of Experience
  48. Femoral Vein Access by Contrast-Guided Puncture in Cardiac Catheterization in Patients Under One Year of Age
  49. Initial Experience With Tadalafil in Pediatric Pulmonary Arterial Hypertension
  50. Balloon Angioplasty for Native Aortic Coarctation in Different Anatomic Variants
  51. Pulmonary Artery Aneurysm in a Child Secondary to Infective Endarteritis
  52. Congenital Complete Atrioventricular Block Associated with QT Prolongation: Description of a Patient with an Unusual Outcome
  53. Incidence of Respiratory Disease During the First Two Years of Life in Children with Hemodynamically Significant Congenital Heart Disease in Italy: A Retrospective Study
  54. Intermediate-Term Results After Extracardiac Conduit Fontan Palliation in Children and Young Adults with Single Ventricle Physiology—A Single-center Experience
  55. Double-Outlet Technique for Tetralogy of Fallot-Type Disease with an Anomalous Coronary Artery
  56. Evaluation of Cardiotoxicity by Tissue Doppler Imaging in Childhood Leukemia Survivors Treated with Low-Dose Anthracycline
  57. Surgical Removal of a Rhabdomyoma in a Neonate as Rescue Therapy
  58. Improved Classification of Coronary Artery Abnormalities Based Only on Coronary Artery z-Scores After Kawasaki Disease
  59. Predicting the Need for Neoaortic Arch Intervention in Infants with Hypoplastic Left Heart Syndrome Through the Glenn Procedure
  60. Lack of Association Between Folate Receptor Autoantibodies and Conotruncal Congenital Heart Defects
  61. Thoracoabdominal Situs: A Practical Approach Accompanied by a Short History of Descriptive Terms
  62. Successful Percutaneous Cardiac Resynchronization Despite an Occlusive Thebesian Valve
  63. Right Aortic Arch, Isolated Left Subclavian Artery and Ductus Arteriosus with Normal Intracardiac Anatomy: Rare Manifestation of Chromosome 22q11 Deletion
  64. A Viable Therapeutic Option: Mechanical Circulatory Support of the Failing Fontan Physiology
  65. Recombinant Factor XIII Reduces Severe Pleural Effusion in Children after Open-Heart Surgery
  66. Autophagy in Cardiac Plasticity and Disease
  67. A Prelude to Fontan
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  69. Stabilizing an Embolized Pre-stent into the Right Ventricle During MELODY Valve Implantation
  70. The Benefit of a General, Systematic Use of Mapping Systems During Electrophysiological Procedures in Children and Teenagers: The Experience of an Adult EP Laboratory
  71. Transient Heart Block in a Neonate Associated with Previously Undiagnosed Maternal Anti-Ro/SSA and Anti-La/SSB Antibodies
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