Cardiovascular Development and Congenital Malformations

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.

Contents

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