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Cardiovascular Development and Congenital Malformations: Molecular & Genetic Mechanisms



Cardiovascular Development and Congenital Malformations: Molecular & Genetic Mechanisms


Congenital cardiovascular malformations are the single most common form of birth defect. Therefore a better understanding of the mechanisms involved in both normal cardiac development and the formation of cardiovascular structural defects is of tremendous importance.

This book brings together the leading scientists from around the world who are actively engaged in studies of the etiology, morphogenesis and physiology of congenital cardiovascular diseases. A broad variety of approaches, techniques, experimental models and studies of human genetics combine to make this a truly outstanding and unique treatise on this pressing topic.

Cardiovascular Development and Congenital Malformations is divided into distinct categories, each focusing on a particular aspect of cardiovascular development. Sections are accompanied by editorial overviews which integrate new findings and place the information into a broader context.

Establishing Left-Right Patterning and Cardiac Looping.

1, Microenvironment provides left–right instructions to migrating precardiac mesoderm.

2, Calmodulin- inv protein interaction and left-right determination.

3, Misexpression of upstream laterality genes on downstream mechanisms of heart looping: A flectin perspective.

4, Pleiotropic effects of Pitx2 isoform c on morphogenesis in the mammalian heart.

5, Signal transductions during cardiac myofibrillogenesis and looping.

6, Biological role of fibulin-2 in cardiovascular development.

Mechanisms of Cardiogenesis and Myocardial Development.

7, TBX5 regulates cardiac cell behavior during cardiogenesis.

8, Cardiac homeobox protein Csx/Nkx2-5and its associated proteins.

9, Regulation of myocardium formation after the initial development of the linear heart tube.

10, The Role of the extracellular matrix (ECM) in cardiac development.

11, Teratogenic effects of bis-diamine on the developing myocardium.

12, Proliferative responses to myocardial remodeling in the developing heart.

Formation of Endocardial Cushions and Valves.

13, TGFâ signaling during atrioventricular cushion transformation.

14, The endocardium as a unique modulator of in utero cardiovascular form and function.

15, Valvulogenesis: Role of periostin in cushion tissue differentiation.

16, Role of Fibroblast growth factors in early valve leaflet formation.

17, Msx1 expression during chick heart development: possible role in endothelial-mesenchymal transformation during cushion tissue formation.

Segment and Chamber Specification.

18, Tbx5 specifies the left/right ventricles and ventricular septum position during cardiogenesis.

19, Transciptional regulation of ventricular morphogenesis.

20, Fgf10 and the embryological origin of outflow tract myocardium.

21, Evolutionary conservation of atrial natriuretic factor (Anf) expression, cardiac chamber formation and the heart-forming region.

Formation of Specialized Conduction Tissues.

22, Induction and Patterning of the Impulse Conducting Purkinje Fiber Network.

23, Spatial Correlation of Conduction Tissue in the Ventricular Trabeculae of the Developing Zebrafish.

24, Development of the cardiac conduction system and contribution of neural crest and epicardially derived cells.

25, The Development of the Cardiac Conduction System: an old story with a new perspective.

26, The role of calreticulin in cardiac development and function.

Coronary Artery Development.

27, Development of proximal coronary artery in quail embryonic heart.

28, Possible roles of the extracellular matrix in coronary vasculogenesis of mouse.

29, Abnormal coronary development in bis-diamine treated embryo.

Models of Congenital Cardiovascular Malformations.

30, Deciphering the basis for congenital heart defects using a mouse model of Holt-Oram syndrome.

31, The role of the transcriptional co-repressor FOG-2 in cardiac development.

32, Molecular mechanisms regulating tissue-specific expression of Tbx1.

33, Tbx1 and DiGeorge syndrome: A genetic link between cardiovascular and pharyngeal development.

34, New insights into the role of Tbx1 in the DiGeorge mouse model.

Role of Neural Crest Cells in Cardiovascular Development.

35, Sdf1/CxcR4 chemotaxisis is disrupted within the splotch mouse mutant cardiovascular system.

36, Differences in migration and differentiation capacities OF early- and late-migrating cardiac neural crest cells.

37, TGF2 does not affect neural crest cell migration but is a keyplayer in vascular remodelling during embryogenesis.

38, Neural Crest Cells Contribute to Heart Formation and Cardiomyogenesis in Zebrafish.

Formation of Outflow Tracts.

39, Outflow tract remodelling – a role for tissue polarity?.

40, Hdf-affected gene fragment revealed by subtraction study of Hdf (heart defect) mouse.

Imaging Techniques.

41, Imaging of heart development in embryos with magnetic resonance microscopy.

Cardiovascular Physiology During Development.

42, Shear stress in the developing cardiovascular system.

43, Quantitative analysis for strech-induced cytoskeletal remodeling in endothelial cells.

44, Differential effects of cyclic stretch on embryonic ventricular cardiomyocyte and non-cardiomyocyte orientation.

45, Physiology and biomechanics of developing cardiovascular systems: Expanding frontiers.

46, Three-dimensional fiber architecture of the embryonic left ventricle during normal development and reduced mechanical load.

47, Changes in cardiac excitation-contraction coupling during mammalian development: Implications for regulation of contractile function.

48, Role of the sodium-calcium exchanger (Ncx- within Splotch (Sp2H) myocardial failure.

49, Sarcolipin, a novel regulator of calcium cycling, is preferentially expressed in the murine and human atrium.

50, Developmental apects of cardiac sensitivity to oxygen deprivation: protective mechanisms in the immature heart.

Patent Ductus Arteriosus.

51, A comprehensive model for O2 constriction in the human ductus arteriosus.

52, Pharmacological Manipulation of the Fetal Ductus Arteriosus in the Rat.

53, Constriction of Ductus Arteriosus by Selective Inhibition of Cyclooxygenase-1 and -2.

54, Synergistic Constriction of Ductus Arteriosus by Indomethacin and Dexamethasone in Fetal Rats.

Human Clinical Genetics and Epidemiology.

55, Prevalence of Congenital Heart Diseases in the Czech Republic.

56, Gene-Environment Interactions in Congenital Heart Disease: An Epidemiological Perspective.

57, Familial Recurrence Risks of Congenital Heart Defects.

58, T-box Gene Family and Congenital Cardiovascular Anomalies.

59, Deciphering the Genetic Etiology of Conotruncal Defects.

60, Cardiovascular Anomalies in Patients with Deletion 22q11.2: A Multi-center Study in Korea.

61, Atrioventricular Canal Defect: Anatomical and Genetic Characteristics.

62, A genetic approach to hypoplastic left heart syndrome.

63, Char syndrome and TFAP2B mutations.

64, The genetic origin of atrioventricular conduction disturbance in humans.

65, Noonan Syndrome and PTPN11 Mutations.

66, Missense Mutations in the PTPN11 as a Cause of Cardiac Defects associated with Noonan Syndrome.

67, Novel Gene Mutations in Patients with Left Ventricular Noncompaction and Evidence for Genetic Heterogeneity.

68, Mitochondrial 16189 DNA Variant and Left Ventricular Hypertrophy in Diabetes Mellitus.

69, Mutation analysis of BMPR2 and other genes in Japanese patients with pediatric primary pulmonary hypertension.

70, Pathophysiology of Williams syndrome arteriopathy

Comprehensive text on the study and care of children with congenital cardiovascular malformations

  • Combines a variety of approaches, techniques, experimental models and studies of human genetics
  • Divided into several sections, each focusing on a particular aspect of cardiovascular development
  • Each section is accompanied by a brief editorial overview to help place the information into a broader context