Molecular Mechanism of Congenital Heart Disease and Pulmonary Hypertension

This open access book focuses on the molecular mechanism of congenital heart disease and pulmonary hypertension, offering new insights into the development of pulmonary circulation and the ductus arteriosus. It describes in detail the molecular mechanisms involved in the development and morphogenesis of the heart, lungs and ductus arteriosus, covering a range of topics such as gene functions, growth factors, transcription factors and cellular interactions, as well as stem cell engineering technologies. The book also presents recent advances in our understanding of the molecular mechanism of lung development, pulmonary hypertension and molecular regulation of the ductus arteriosus. As such, it is an ideal resource for physicians, scientists and investigators interested in the latest findings on the origins of congenital heart disease and potential future therapies involving pulmonary circulation/hypertension and the ductus arteriosus.

Discusses the latest concepts regarding the molecular mechanism of congenital heart disease Includes contributions by an international group of distinguished scientists and clinicians Highlights the latest research on pulmonary hypertension, with a focus on clinical implications and future directions

Autorentext

Toshio Nakanishi

Tokyo Women's Medical University

Department of Pediatric Cardiology

Tokyo, Japan

nakanishi.toshio@twmu.ac.jp

Scott Baldwin

Vanderbilt University Medical Center

Department of Pediatrics (Cardiology) and Cell and Development Biology

Nashville

Tennessee, USA

scott.baldwin@Vanderbilt.Edu

Jeffrey Fineman

University of California

UCSF Benioff Children's Hospital

San Francisco

California, USA

Jeff.Fineman@ucsf.edu

Hiroyuki Yamagishi

Keio University School of Medicine

Department of Pediatrics

Shinjyuku-ku

Tokyo, Japan hyamag@keio.jp

Inhalt
PART I: Basic Science of Pulmonary Development and Pulmonary Arterial Disease.- 1 Perspective for Part I.- 2 The alveolar stem cell niche of the mammalian lung.- 3 Lung development and Notch signalling.- 4 Specialized smooth muscle cell progenitors in pulmonary hypertension.- 5 Diverse Pharmacology of Prostacyclin Mimetics: Implications for Pulmonary Hypertension.- 6 Endothelial-to-mesenchymal transition in pulmonary hypertension.- 7 Extracellular vesicles, MicroRNAs and Pulmonary Hypertension.- 8 Roles of Tbx4 in the lung mesenchyme for airway and vascular development.- 9 A lacZ reporter transgenic mouse line revealing the development of pulmonary artery.- 10 Roles of stem cell antigen-1 in the pulmonary endothelium.- 11 Morphological characterization of pulmonary microvascular disease in bronchopulmonary dysplasia caused by hyperoxia in newborn mice.- 12 Involvement of CXCR4 and stem cells in a rat model of pulmonary arterial hypertension.- 13 Ca2+ signal through inositol trisphosphate receptors for cardiovascular development and pathophysiology of pulmonary arterial hypertension.- PART II: Abnormal pulmonary circulation in the developing lung and heart.- 14 Perspective for Part II.- 15 Pathophysiology of Pulmonary Circulation in Congenital Heart Disease.- 16 Development of Novel Therapies for Pulmonary Hypertension by Clinical Application of Basic Research.- 17 Using Patient-Specific Induced Pluripotent Stem Cells to Understand and Treat Pulmonary Arterial Hypertension.- 18 Modeling pulmonary arterial hypertension using induced pluripotent stem cells.- 19 Dysfunction and restoration of endothelial cell communications in Pulmonary Arterial Hypertension: Therapeutic implications.- 20 Inflammatory Cytokines in the Pathogenesis of Pulmonary Arterial Hypertension.- 21 Genotypes and Phenotypes of Chinese Pediatric Patients with Idiopathic and Heritable Pulmonary Arterial Hypertension- Experiences from A Single Center.- 22 Fundamental Insight into Pulmonary Vascular Disease : Perspectives from Pediatric PAH in Japan.- 23 Risk stratification in paediatric pulmonary arterial hypertension.- 24 The Adaptive Right Ventricle in Eisenmenger Syndrome: Potential Therapeutic Targets for Pulmonary Hypertension.- 25 Impaired right coronary vasodilator function in pulmonary hypertensive rat assessed by in vivo synchrotron microangiography.- 26 Relationship between mutations in ENG and ALK1 gene and the affected organs in hereditary hemorrhagic telangiectasia.- 27 A genetic analysis for patients with pulmonary arterial hypertension.- 28 Evaluation and visualization of right ventricle using three dimensional echocardiography.- 29 Pulmonary hypertension associated with post-operative Tetralogy of Fallot.- 30 Microscopic Lung Airway Abnormality and Pulmonary Vascular Disease Associated with Congenital Systemic to Pulmonary Shunt.- 31 Respiratory syncytial virus infection in infants with heart and lung diseases.- PART III: Ductus arteriosus: bridge overtroubled vessels.- 32 Perspective for Part III.- 33 The ductus arteriosus, a vascular outsider, in relation to the pulmonary circulation.- 34 Molecular, genetic, and pharmacological modulation of the ductus arteriosus: KATP channels as novel drug targets.- 35 New mediators in the biology of the ductus arteriosus: Lessons from the chicken embryo.- 36 Constriction of the Ductus Arteriosus with KATP Channel Inhibitors.- 37 New insights on how to treat patent ductus arteriosus.- 38 Antenatal Administration of Betamethasone Contributes to Intimal thickening of the Ductus Arteriosus.- 39 Prostaglandin E-EP4-mediated fibulin-1 up-regulation plays a role in intimal thickening of the ductus arteriosus.- 40 Transcriptional profiles in the chicken ductus arteriosus during hatching.- 41 Inhibition of Cyclooxygenase Contracts Chicken Ductus Arteriosus.- 42 Prostaglandin E2 receptor EP4 inhibition constricts the rat ductus arteriosus.- 43 Dilatation of the Ductus Arteriosusby Diazoxide in Fetal and Neonatal Rats.- 44 The Effect of Long-term Administration of Plostaglandin E1 on Morphological Changes in Ductus Arteriosus.- 45 Significance of SGK1 as a protein kinase transcriptionally regulated by ALK1 signaling in vascular endothelial cells.- 46 Fabrication of Implantable Human Arterial Graft by Periodic Hydrostatic Pressure.- 47 Optimum preparation of Candida albicans cell wall extra (CAWE) for the mouse model of Kawasaki disease.- PART IV: Development and Regeneration of the Cardiovascular System.- 48 Perspective for Part IV.- 49 Advances in the second heart field.- 50 Novel cardiac progenitors for all components of the heart except for the right ventricle.- 51 Regional and TBX5-dependent gene expression in the atria: Implications for pulmonary vein development and atrial fibrillation.- 52 The Endocardium as a Master Regulator of Ventricular Trabeculation.- 53 The Role of Alternative mRNA Splicing in Heart Development.- 54 Progress in the Generation of Multiple Lineage Human-iPSC-derived 3D Engineered Cardiac Tissues for Cardiac Repair.- 55 Quantification of contractility in stem cell derived cardiomyocytes.- 56 A neurotrophic factor receptor GFRA2, a specific surface antigen for cardiac progenitor cells, regulates the process of myocardial compaction.- 57 Cardiac cell specification and differentiation by the defined factors.- 58 A Temporo-Spatial Regulation of Sema3c is Essential for Interaction of Progenitor Cells during Cardiac Outflow Tract Development.- 59 Spatiotemporally restricted developmental alterations in the anterior and secondary heart fields cause distinct conotruncal heart defects.- 60 Significance of transcription factors in the mechanisms of great artery malformations.- 61 The different c-kit expression in human induced pluripotent stem (iPS) cells between with feeder cells and without feeder cells.- 62 Establishment of induced pluripotent stem cells from immortalized B cell lines and their differentiation into cardiomyocytes.- 63 Establishment of an in vitro LQT3 model, using induced pluripotent stem cells from LQT3 patient-derive…