Molecular Mechanism of Congenital Heart Disease and Pulmonary Hypertension

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 signaling 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 over troubled 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 Arteriosus by 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-derived cardiomyocytes 64 Genetic Assessments for clinical courses of Left ventricle noncompaction 65 Elucidating the pathogenesis of congenital heart disease in the era of next-generation sequencing.

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.

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