Pediatric Cardiology - Fetal, Pediatric, and Adult Congenital Heart Diseases (Sep 6, 2024)_(3031075625)_(Springer).pdf

Foreword Preface Contents About the Editor-in-Chief About the Editors About the Section Editors Managing Editor Contributors Part I: Introduction 1 Pediatric Cardiology: A Journey to the Past 1 Introduction 2 Evolution of Recognizing Cardiovascular Structure and Function 3 The Initial Phases of Pediatric Cardiology 4 Evolution of Electrocardiography 5 Echocardiography: To See Through Sound 6 The History of Medical Literature and Pediatric Cardiology 7 Cardiac Catheterization: The Man Was Daring! 8 Rheumatic Heart Disease: Once the Dominant Lesion in Pediatric Cardiology 9 Kawasaki Disease: Taking Over Rheumatic Heart Disease 10 Training in Pediatric Cardiology 10.1 The Founders 10.2 Governing Bodies 10.3 Changing Training Requirements References 2 Normal Development of the Heart 1 Introduction 2 Initial Formation of the Embryo 3 Looping of the Heart Tube 4 Formation and Separation of the Atrial Chambers 5 Formation and Septation of the Atrioventricular Canal 6 Formation of the Ventricles 7 Formation and Septation of the Aortic and Pulmonary Pathways 8 Development of the Cardiac Conduction System 9 Development of the Coronary Circulation 10 Development During the Fetal and Neonatal Periods References 3 Normal Cardiac Anatomy 1 Introduction 2 Attitudinally Appropriate Descriptions 3 The Cardiac Seat Belt 4 The Cardiac Chambers 4.1 The Morphologically Right Atrium 4.2 The Morphologically Left Atrium 4.3 The Morphologically Right Ventricle 4.4 The Morphologically Left Ventricle 5 The Arterial Roots and Intrapericardial Trunks 6 The Atrioventricular and Arterial Valvar Complexes 6.1 The Atrioventricular Valvar Complexes 6.2 The Arterial Valvar Complexes 7 The Septal Structures 8 The Fibrous Skeleton 9 The Conduction System 10 The Blood Supply to the Heart 10.1 The Coronary Arteries 10.2 The Coronary Veins References 4 Sequential Segmental Analysis of Cardiac Morphology 1 Introduction 2 Sequential Segmental Analysis 3 Arrangement of the Organs 4 Atrial Assessment 5 Determination of the Atrioventricular Connections 6 Types of Atrioventricular Connections 7 Types of Atrioventricular Valves 8 Ventricular Morphology 9 Ventricular Topology 10 Assessment of the Ventriculo-Arterial Junctions 10.1 Infundibular Relationships 10.2 Arterial Relationships References 5 The Role of Genetics in Development of Congenital Heart Disease 1 Introduction 2 Epidemiology and Etiology of CHD 2.1 Incidence 2.2 Prevalence According to Disease Type 3 Etiologies of CHD 3.1 Genetic Factors 3.1.1 Chromosomal Abnormalities 3.1.2 Monogenic Diseases 3.1.3 Copy Number Variation (CNV) 3.2 Environmental Factors 3.3 Multifactorial Inheritance 3.4 Recurrence 3.4.1 Multifactorial Inheritance 3.4.2 Chromosomal and Genetic Abnormalities 3.5 Genetic Counseling 4 Genetic Disorders Associated with CHD 4.1 Down Syndrome 4.1.1 Prevalence 4.1.2 Clinical Features 4.1.3 Heart Disease in Down Syndrome 4.1.4 Genetics 4.2 Trisomy 18 4.2.1 Prevalence 4.2.2 Clinical Features 4.2.3 Heart Disease in Trisomy 18 4.2.4 Genetics 4.3 Trisomy 13 4.3.1 Prevalence 4.3.2 Clinical Features 4.3.3 Heart Disease in Trisomy 13 4.3.4 Genetics 4.3.5 Prognosis and Treatment of Trisomy 18 and 13 4.4 Turner Syndrome 4.4.1 Prevalence 4.4.2 Clinical Features 4.4.3 Heart Disease in Turner Syndrome 4.4.4 Genetics 4.5 22q11.2 Deletion Syndrome 4.5.1 Prevalence 4.5.2 Clinical Features 4.5.3 Heart Disease in 22q11.2 Deletion Syndrome 4.5.4 Genetics 4.6 Williams Syndrome 4.6.1 Prevalence 4.6.2 Clinical Features 4.6.3 Heart Disease in Williams Syndrome 4.6.4 Genetics 4.7 Noonan Syndrome 4.7.1 Prevalence 4.7.2 Clinical Features 4.7.3 Heart Disease in Noonan Syndrome 4.7.4 Genetics 4.8 Marfan Syndrome 4.8.1 Prevalence 4.8.2 Clinical Features 4.8.3 Heart Disease in Marfan Syndrome 4.8.4 Genetics 4.9 CHARGE Syndrome 4.9.1 Prevalence 4.9.2 Clinical Features 4.9.3 Heart Disease in CHARGE Syndrome 4.9.4 Genetics 4.10 Holt-Oram Syndrome 4.10.1 Prevalence 4.10.2 Clinical Features 4.10.3 Heart Disease in Holt-Oram Syndrome 4.10.4 Genetics 4.11 Genetic Abnormalities of Nonsyndromic CHD (Table 4) [81-83] 4.11.1 NKX2.5 4.11.2 GATA4 and GATA6 4.11.3 NOTCH1, NOTCH2, and JAG1 4.11.4 TBX20 5 Recent Advances in the Role of Genetics in CHD 5.1 The Role of Genetics in Heterotaxy Syndrome 5.1.1 Genetic Etiology of Heterotaxy Syndrome 5.2 The Role of Genetics in the Outflow Tract Defects 5.2.1 Identification of a Genetic Etiology of the Outflow Tract Defects 5.2.2 Molecular Mechanisms Underlying the Outflow Tract Development: Interaction Between SHF Cells and CNC Cells 6 Concluding Remarks References 6 Teratogenic Factors Contributing to the Development of Congenital Heart Defects 1 Introduction 2 Understanding Risk Factor Studies 2.1 Understanding Measurements of Associations in Risk Factor Studies of Congenital Heart Defects 2.2 Challenges of Studying Risk Factors for Congenital Heart Defects 2.3 Databases of Patients with Congenital Heart Defects 3 Select Risk Factors 3.1 Maternal Sociodemographic Characteristics 3.1.1 Maternal Age 3.1.2 Maternal Reproductive History 3.1.3 Maternal Stress 3.1.4 Socioeconomic Status 3.2 Maternal Health Conditions 3.2.1 Diabetes Mellitus 3.2.2 Epilepsy 3.2.3 Febrile Illnesses: Upper Respiratory Infection, Genitourinary Infections, and Influenza 3.2.4 Hypercholesterolemia and Atherosclerotic Heart Disease 3.2.5 Hyperhomocysteinemia 3.2.6 Hypertension 3.2.7 Phenylketonuria 3.2.8 Pre-Pregnancy Weight 3.2.9 Maternal Rubella Infection 3.3 Maternal Therapeutic Medications 3.3.1 Antiasthmatics 3.3.2 Antibiotics 3.3.3 Antidepressant Medications 3.3.4 Antiepileptics 3.3.5 Antifungals 3.3.6 Antihypertensives 3.3.7 Anti-Inflammatories 3.3.8 Folic Acid 3.4 Maternal Nontherapeutic Drugs 3.4.1 Alcohol 3.4.2 Cigarette Smoking 3.4.3 Illicit Drugs 3.5 Maternal Environmental Exposures 3.5.1 Air Pollution 3.5.2 Chemical Exposures 3.5.3 Water Contamination 4 Conclusion References 7 Ethical Considerations in Fetal and Pediatric Cardiology 1 Introduction 1.1 Ethics and Pediatric Cardiology 1.2 Ethics, the Discipline 1.3 Ethical Theories and Conceptual Frameworks 1.4 Value Plurality and Culture 2 Ethics in Practice 2.1 Informed Consent 2.2 Child and Adolescent Decision-Making 2.3 Best Interest Standard 2.4 Shared Decision-Making 2.5 Ethical Controversies in Non-intervention 2.6 Ethical Controversies in Intervention 2.7 Extracardiac and Genetic Anomalies 2.8 Pediatric Heart Transplantation 2.8.1 Use of Resources and Organ Allocation 2.8.2 Informed Consent in Transplant 2.8.3 Mechanical Support 2.8.4 Psychosocial Factors 2.8.5 Quality of Life and End-of-Life Care 2.8.6 Regulatory Aspects of Organ Donation and Transplantation 2.9 Use of Ethics Consultation Services 3 Ethics in a Changing World 3.1 Transparency and Regionalization in Pediatric Cardiology and Cardiac Surgery 3.2 Disparities in Care and Social Determinants of Health 3.3 Global Perspective in Congenital Heart Disease 3.4 Innovation in Cardiology and Cardiac Surgery 3.4.1 Innovation Versus Research 3.4.2 New Practices Versus First Practices 4 Conclusion References 8 Quality and Value Improvement in Pediatric Cardiac Care 1 Quality Improvement Background 1.1 Donabedian: Structure, Process, and Outcome 1.2 Institute of Medicine Quality Domains 1.3 Improvement Frameworks: Model for Improvement, Six Sigma, and Lean 1.4 Quality Improvement Tools 1.5 Quality Improvement Versus Research (and Accountability) 2 Safety 2.1 Categorizing Safety 2.2 Measuring Safety 2.3 A Global and Cultural Framework for Safety 2.4 The Future of Safety in Health Care 3 Healthcare Value 3.1 The Formula for Healthcare Value 3.2 The Outcomes Hierarchy 3.3 True Cost 3.4 Healthcare Value and Health Equity 3.5 A Proposed Quality-Value Relationship 4 Variation and Standardization 4.1 Research Studies Characterizing Variation 4.2 Measure Development and Variation Reduction Through Collaboratives 4.3 Improving Quality Through Standardization 5 Learning from Data in QI 5.1 Learning from Data over Time 5.2 Variation and Statistical Quality Improvement Methods 5.3 Run Charts and Statistical Process Control (SPC): A Simple Example 6 External Forces as Drivers of Improvement Efforts 6.1 U.S. News & World Report Best Children´s Hospital Rankings 6.2 Conquering CHD 7 Local Improvement Structure and Lessons Learned: The Improvement Journey of Two Unique Pediatric Cardiac Programs 8 Conclusions References 9 Research in Pediatric Cardiology 1 Pediatric Cardiology and Clinical Research 2 Quality and Safety in Pediatric Care: Overcoming the Implementation Gap 2.1 Coproduction of Optimal Pediatric Outcomes 2.2 Bringing High Reliability into Pediatric Healthcare 2.3 Enhancing Learning and Improvement Within the Cardiology Microsystem 2.4 A Human Factors-Social-Technical Systems Approach to Healthcare Quality and Patient Safety 2.5 Reducing Diagnostic Errors in Pediatric Cardiology 2.6 Safety and Quality Outcome Measurement and Monitoring 2.7 The Science of Continuous Quality and Performance Improvement 2.8 The Essential Role Teamwork Plays in Enabling Pediatric Safety and Quality 2.9 The Role of Communication and Coordination in Improving Safety and Quality Team Performance 2.10 Turning Healthcare Experts into an Expert Team Requires Substantial Planning and Practice 2.11 The Learning Organization: Capturing Process and Outcomes Failures While Engaging Staff 2.12 Creating a Culture of Trust: The Cornerstone of Clinician and Management Engagement for Safety 2.13 Implementation Science and Approaches to Overcome Unseen Barriers to Change 2.14 Regionalization Learning Collaboratives of Pediatric Cardiac Services 2.15 The Future of Quality Improvement and Culture Change in Pediatrics 3 Future Directions References 10 Fetal Autopsy and Cardiac Registry 1 Introduction 2 Interdepartmental Communication 3 Autopsy Permit 4 The Autopsy 5 Dilation and Evacuation Specimens 6 Cardiac Collections 7 Advancing Modern Collections References 11 Worldwide Practice of Pediatric Cardiology 1 Introduction 2 Challenges in Organizing Cardiac Care in Low- and Low-Middle-Income Countries 2.1 Financial Support 2.2 Access to Care and Poor Awareness 2.3 Education and Training 2.4 Research 2.5 Experience from Local Programs and More Global Evaluations 3 Challenges and Success: Review of Programs 3.1 Mozambique 3.2 Guatemala 3.3 South Africa 3.4 Pakistan 3.5 East Africa 3.6 Lebanon 4 Global Evaluation of Local Programs in LMI Countries 5 Building a Regional Cardiac Program in Low- and Low-Middle-Income Regions: Lessons Learned from International Experts and Pe... 5.1 Twinning Partnership 5.2 A Plan 5.3 Reconnaissance 5.4 Selection of Local Partners 5.5 Continuous Support 5.6 Building a Team Coupled with Continuous Education 5.7 Quality Control 5.8 Equipment 5.9 Local Solutions 5.10 Starting the Program 5.11 Communication with the Community 5.12 Locally Generated Research 6 Conclusion 7 Testimonials 8 Reflections on a Personal Journey to Provide Cardiac Care in Namibia: By Christopher Hugo-Hamman 8.1 Background 8.2 Evolution 8.3 The Culture of Pediatric Cardiac Care 8.4 Human Resources Constraints 8.5 Infrastructure 8.6 Management Systems 8.7 Political Will 9 Testimony of an NGO Director: Steve Sosebee, President of Palestine Children´s Relief Fund (PCRF) 10 Testimony: Experience of a Pediatric Cardiologist in Rwanda by Emmanuel Rusingiza 10.1 Personal Background 10.2 The State of Pediatric Cardiac Diseases in Public Setting and Access to Care 10.3 Current State of Cardiac Diseases in Rwanda 10.4 Hope for Unmet Ambitions 11 Quotes by Christopher Hugo-Hamman (CHH), Steve Sosebee (StS), Emmanuel Rusingiza (ER), and Daniel De Wolf (DDW) References Part II: Fetal Cardiology 12 Fetal Cardiovascular Physiology 1 Introduction 2 Normal Fetal Circulation 2.1 Course of Blood Flow in the Normal Fetal Circulation 3 The Venous Duct 4 The Oval Foramen 5 The Arterial Duct 5.1 Histology of the Arterial Duct 5.2 Postnatal Ductal Closure 5.3 The Role of the Arterial Duct (AD) in the Presence of CHD 6 Fetal Cardiac Output and Circulatory Regulation 7 The Baroreflex and Chemoreflex in the Fetus 8 The Fetal Myocardium 9 Gas Exchange and O2 Delivery 10 Role of Fetal Hemoglobin 11 Postnatal Circulation 12 Change in Cardiac Output, Hemoglobin, and Myocardium 13 Assessment of Fetal Myocardial Performance References 13 Fetal Echocardiography 1 Background 2 Indications 3 Fetal Echocardiogram 3.1 Cardiac Structure Fig. 10 Standard fetalechoshortaxisviews 3.2 Hemodynamic Assessment Fig. 11 Great vesselshortaxisview. RV right ventricle,Ao aortic valve, PA pulmonary artery 3.2.1 Umbilical Vessels Fig. 12 Umbilical arteryandumbilicalveinDoppler flow 3.2.2 Systemic Veins Fig. 13 Ductus venosus flow. S ventricular systole, D diastole, A atrial systole Fig. 14 Hepatic veinDoppler. S ventricular systole, D diastole, A atrial systole 3.2.3 Pulmonary Veins Fig. 15 Right pulmonaryveinDoppler. S ventricular systole, D diastole, A atrial systole 3.2.4 Atrioventricular Valves Fig. 16 Tricuspidvalveinflow Doppler. E early diastole, A atrial systole 3.2.5 Semilunar Valves 3.2.6 Foramen Ovale Fig. 17 Aortic outflow Doppler.Peakvelocity60cm/s 3.2.7 Aortic Arch 3.2.8 Arterial Ducts 3.3 Rhythm Assessment 3.4 Function Assessment References 14 Fetal Cardiovascular MRI 1 Introduction 2 Rationale for the Development of Fetal Cardiovascular MRI 2.1 Accuracy of Prenatal Diagnosis of CHD 2.2 Longitudinal Monitoring of Fetal Cardiac Treatment and Interventions 2.3 Impairment of Prenatal Brain Development in the CHD Population 2.4 The Role of Placental Disease in CHD Fetuses 3 Safety 4 Technical Aspects of Fetal Cardiovascular MRI 5 Magnetic Resonance Oximetry 6 Improving the Fetal Diagnosis of CHD Using Fetal Cardiovascular MRI 7 Role of Cardiovascular MRI in Understanding Fetal Cardiovascular Physiology 7.1 The Normal Circulation 7.2 Intrauterine Growth Restriction 7.3 Acute Maternal Hyperoxygenation 7.4 Pathophysiology of Congenital Heart Diseases (CHD) 8 Assessment of the Impact of Fetal Interventions by Fetal Cardiovascular MRI 8.1 Invasive Fetal Cardiac Interventions 8.2 Fetal Anemia and Transfusions 8.3 Pharmacological Cardiac Interventions 9 Utility of Fetal Cardiovascular MRI to Investigate the Neurodevelopmental Impact of CHD and IUGR 10 Potential Role of Fetal Cardiovascular MRI for the Assessment of Placental Function 11 How to Perform a Fetal Cardiac MRI: Practical Aspects and Illustrative Cases 12 Current Limitations 13 Future Perspectives 14 Conclusion References 15 Fetal Systemic Venous Anomalies 1 Introduction 2 Superior Caval Vein Anomalies 2.1 Persistent Left Superior Caval Vein to an Intact Coronary Sinus 2.1.1 Morphogenesis 2.1.2 Prevalence and Associated Defects 2.1.3 Fetal Echocardiography 2.1.4 Clinical Implications and Management 2.1.5 Genetics 2.2 Persistent Left Superior Caval Vein to an Unroofed Coronary Sinus 2.2.1 Morphogenesis 2.2.2 Prevalence and Associated Defects 2.2.3 Fetal Echocardiography 2.2.4 Clinical Implications and Management 2.2.5 Genetics 2.3 Left Superior Caval Vein to Left Atrium 2.3.1 Morphogenesis 2.3.2 Prevalence 2.3.3 Fetal Echocardiography 2.3.4 Clinical Implications and Management 2.4 Right SVC to Left Atrium 2.4.1 Morphogenesis 2.4.2 Prevalence 2.4.3 Fetal Echocardiography 2.4.4 Clinical Implications and Management 3 Inferior Caval Vein Anomalies 3.1 Interrupted Inferior Caval Vein with Azygous or Hemiazygous Continuation 3.1.1 Morphogenesis 3.1.2 Prevalence and Associated Defects 3.1.3 Fetal Echocardiography 3.1.4 Clinical Implications and Management 3.2 Bilateral Inferior Caval Veins 3.2.1 Morphogenesis 3.2.2 Prevalence and Associated Defects 3.2.3 Clinical Implications and Management 3.3 Left Inferior Caval Vein to Right Atrium (Absent Right Inferior Caval Vein) 3.3.1 Morphogenesis 3.3.2 Prevalence 3.3.3 Clinical Implications and Management 3.4 Agenesis of the Ductus Venosus 3.4.1 Morphogenesis 3.4.2 Prevalence and Associated Defects 3.4.3 Fetal Echocardiography 3.4.4 Clinical Implications and Management 3.4.5 Genetics References 16 Fetal Pulmonary Venous Anomalies 1 General Introduction 2 Total Anomalous Pulmonary Venous Return (TAPVR) 2.1 Introduction: Prevalence, Embryology, and Associated Lesions 2.2 Prenatal Ultrasound Manifestations 2.3 Prenatal Ultrasound Findings in TAPVR Types 2.3.1 Supracardiac TAPVR 2.3.2 Infracardiac TAPVR 2.3.3 Cardiac TAPVR 2.3.4 Mixed Type Prenatal Follow-Up and Delivery Preparation 2.4 Prenatal Counseling 3 Partial Anomalous Pulmonary Venous Return (PAPVR) 3.1 Introduction: Prevalence, Embryology, and Associated Lesions 3.2 Prenatal Ultrasound Manifestations 3.3 Prenatal Counseling References 17 Fetal Ebstein Malformation 1 Introduction 2 Morphology and Associated Anomalies 3 Associated Anomalies 4 Manifestation on Obstetrical Ultrasound 5 Antenatal History and Role of Genetics 6 Fetal Echocardiography 7 Fetal Life Follow-Up, What to Expect 8 Interventions 9 Hemodynamic Changes at Birth 10 Preparation at Delivery and Immediate Postnatal Care 10.1 Cardiac Function 10.2 Tricuspid Insufficiency 10.3 Pulmonary Flow 10.4 Presence of Arrhythmia 10.5 Lung Hypoplasia or Presence of Other Malformations 10.6 Presence of Hydrops 10.7 Neonatal Surgical Intervention References 18 Fetal Pulmonary Stenosis 1 Introduction 2 Classification 3 Associated Cardiac Findings in Pulmonary Stenosis 3.1 Poststenotic Dilatation of the Main Pulmonary Artery 3.2 Nondilated Main Pulmonary Artery in Pulmonary Stenosis 3.3 Tricuspid Valve Morphology, Size, and Function 3.4 Right Ventricular Morphology and Function 3.5 Right Atrial Dilatation 3.6 Reversal of Flow in the Arterial Duct 3.7 Ventriculocoronary Arterial Communication 4 Natural History 5 Fetal and Neonatal Management References 19 Fetal Left Heart Obstructive Lesions: Aortic Stenosis, Shone´s Complex, and Aortic Arch Obstruction 1 Aortic Valve Stenosis 1.1 Introduction 1.2 Manifestation of Lesion in OB Ultrasound 1.3 History Taking 1.4 Details of Fetal Echocardiography 1.5 Fetal Life Follow-Up 1.6 Interventions 1.7 Expected Changes in Hemodynamics at Birth 1.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 1.9 Parental Counseling 2 Coarctation of the Aorta 2.1 Introduction 2.2 Manifestations of Lesion in OB Ultrasound 2.3 History Taking and Additional Testing 2.4 Details of Fetal Echocardiography 2.5 Fetal Life Follow-Up 2.6 Interventions 2.7 Expected Changes in Hemodynamics at Birth 2.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 2.9 Parental Counseling 3 Shone´s Complex 3.1 Introduction 3.2 Manifestations of Lesion in OB Ultrasound 3.3 History Taking 3.4 Details of Fetal Echocardiography 3.5 Fetal Life Follow-Up 3.6 Interventions 3.7 Expected Changes in Hemodynamics at Birth 3.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 3.9 Parental Counseling 4 Interrupted Aortic Arch 4.1 Introduction 4.2 Manifestations of Lesion in OB Ultrasound 4.3 History Taking 4.4 Details of Fetal Echocardiography 4.5 Fetal Life Follow-Up 4.6 Interventions 4.7 Expected Changes in Hemodynamics at Birth 4.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 4.9 Parental Counseling References 20 Fetal Hypoplastic Left Heart Syndrome 1 Introduction 2 Cardiac Anatomy in Hypoplastic Left Heart Syndrome and Anatomic Associations 3 Incidence and Genetics 4 Prenatal Detection of HLHS 5 Evolving HLHS: Possible Mechanisms and Prenatal Findings 6 Cardiac Structure and Fetal Echocardiographic Features in HLHS 6.1 Systemic Venous Return and Right Atrium 6.2 Atrial Septum and Oval Foramen 7 Pulmonary Veins 8 Levo-atrial Cardinal Vein 9 Mitral Valve 9.1 Left Ventricle 9.2 Aortic Valve 10 Aortic Arch 11 Evaluation of the RV 12 Tricuspid Valve Morphology and Function 13 Cerebral Blood Flow Pattern in HLHS Fetus 14 Impact of Prenatal Diagnosis of HLHS 15 Delivery Planning for a Fetus with HLHS 16 Parental Counseling References 21 Fetal Transposition of the Great Arteries 1 Transposition 1.1 Introduction and Prevalence 1.2 Morphology 1.2.1 Prenatal Genetic Testing 1.3 Pathophysiological Observations in TGA During Prenatal Life 1.4 Prenatal Maternal Hyperoxia 1.5 Echocardiographic Findings 1.6 Transabdominal Imaging 1.7 Associated Defects 1.8 Differential Diagnosis 1.9 Fetal Life Follow-Up, What to Expect 1.10 Hemodynamic Changes at Birth and Clinical Presentation 1.11 Outcome and Prognosis 2 Corrected Transposition 2.1 Introduction and Prevalence 2.2 Associated Defects 2.3 Manifestation in Obstetrical Ultrasound 2.4 Prenatal Genetic Testing 2.5 Fetal Echocardiography 2.6 Fetal Life Follow-Up, What to Expect 2.7 Outcome and Prognosis 2.8 Postnatal Management 3 Conclusion References 22 Fetal Conotruncal Defects 1 Introduction 1.1 Genetics of Conotruncal Defects 2 Tetralogy of Fallot 2.1 Introduction 2.2 Anatomy and Physiology 2.3 Fetal Diagnosis 2.4 Fetal Echocardiography 2.5 Special Fetal Imaging Considerations 2.6 Outcomes 2.7 Fetal Follow-Up and Counseling 2.8 Delivery Planning 3 Common Arterial Trunk 3.1 Anatomy and Physiology 3.2 Fetal Diagnosis 3.3 Fetal Echocardiography 3.4 Fetal Follow-Up and Counseling 3.5 Delivery Planning 4 Double-Outlet Right Ventricle 4.1 Anatomy and Physiology 4.2 Fetal Diagnosis 4.3 Fetal Echocardiography 4.4 Outcomes 4.5 Fetal Follow-Up and Counseling 4.6 Delivery Planning References 23 Fetal Functionally Single Ventricle 1 Pulmonary Atresia with Intact Ventricular Septum 1.1 Introduction 1.2 Manifestation in Obstetrical Ultrasound and History Taking 1.3 Details of Fetal Echocardiography 1.4 Fetal Follow-Up 1.5 Fetal Interventions 1.6 Delivery Preparation and Postnatal Outcome 2 Unbalanced Atrioventricular Canal 2.1 Introduction 2.2 Manifestation in Obstetrical Ultrasound and History Taking 2.3 Details of Fetal Echocardiography 2.4 Fetal Follow-Up 2.5 Delivery Preparation and Postnatal Outcome 3 Tricuspid Atresia 3.1 Introduction 3.2 Manifestation in Obstetrical Ultrasound and History Taking 3.3 Details of Fetal Echocardiography 3.4 Fetal Follow-Up 3.5 Delivery Preparation and Postnatal Outcome 4 Double-Inlet Left Ventricle 4.1 Introduction 4.2 Manifestation in Obstetrical Ultrasound and History Taking 4.3 Details of Fetal Echocardiography 4.4 Fetal Follow-Up 4.5 Delivery Preparation and Postnatal Outcome References 24 Fetal Vascular Ring 1 Introduction 1.1 Normal Arch Embryology and Identification in Fetal Echocardiography 2 Right-Sided Aortic Arches and Subtypes 2.1 Right Aortic Arch with Mirror Image Branching 2.2 Right Aortic Arch with an Aberrant Left Subclavian Artery 2.3 Other Right Aortic Arch Variants 3 Double Aortic Arch 4 Alternative Imaging for RAA and DAA 5 Left Pulmonary Artery (LPA Sling) 6 Prenatal Counseling References 25 Fetal Hydrops and Heart Failure 1 Introduction 2 Pathophysiology 3 Etiology 3.1 Structural Cardiac Abnormalities 3.2 Cardiac Failure 3.3 Arrhythmias 3.4 High-Output Failure 3.5 Idiopathic Arterial Calcification 3.6 Genetic Etiologies 3.7 Twin-Twin Transfusion and TRAP Sequence 4 Diagnostic Evaluation 5 Evaluation by Fetal Echocardiogram 6 Fetal Interventions 7 Preparation for Delivery and Immediate Postnatal Care 8 Outcomes References 26 Fetal Arrhythmia: Bradycardia and Tachycardia 1 Introduction 2 Echocardiographic Assessment of Fetal Rhythm 2.1 2D Assessment of Fetal Heart Rhythm 2.2 M-Mode Assessment of Fetal Heart Rhythm 2.3 Spectral Doppler Assessment of Fetal Heart Rhythm 2.4 Tissue Doppler Assessment of Fetal Heart Rhythm 2.5 Fetal Magnetocardiography Assessment of the Fetal Heart Rhythm 3 Types of Arrhythmias 3.1 Irregular Heart Rhythm 3.1.1 Premature Atrial Contractions 3.1.2 Premature Ventricular Contractions 3.1.3 Second-Degree AV Block 3.2 Tachyarrhythmias 3.2.1 Sinus Tachycardia 3.2.2 Reentrant Supraventricular Tachycardia 3.2.3 Treatment of SVT 3.2.4 Treatment of SVT in the Hydropic Fetus 3.2.5 Atrial Flutter 3.2.6 Treatment of Atrial Flutter 3.2.7 Ventricular Tachycardia 3.2.8 Rare Fetal Tachyarrhythmias 3.3 Bradyarrhythmia 3.3.1 Sinus Bradycardia 3.3.2 Complete Heart Block 3.3.3 Treatment of Complete Heart Block 3.3.4 Blocked Atrial Bigeminy 4 Channelopathies 5 Conclusions References 27 Fetal Cardiac Intervention 1 Introduction 2 Severe Mid-gestation Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome 2.1 Patient Selection 2.2 Technical Aspects and Procedural Success 2.3 Postnatal Outcome 3 Pulmonary Atresia with Intact Ventricular Septum or Critical Pulmonary Stenosis and Evolving Hypoplastic Right Heart Syndrome 3.1 Patient Selection 3.2 Technical Aspects and Procedural Success 3.3 Postnatal Outcome 4 Established Hypoplastic Left Heart Syndrome with Intact or Highly Restrictive Atrial Septum 4.1 Patient Selection 4.2 Technical Aspects and Procedural Success 4.3 Postnatal Outcome 5 Less Commonly Performed Fetal Cardiac Interventions 6 Future Directions References 28 Delivery Planning for Complex Congenital Heart Disease 1 Introduction 2 Fetal Circulation and Transitional Physiology 3 In Utero Progression of CHD 4 Principles for Delivery Planning 4.1 A Multidisciplinary Approach for Delivery Planning 4.2 Choosing Appropriate Delivery Location Based on ``Levels of Neonatal Care´´ 4.3 Classification of CHD Severity Based on Fetal Echocardiographic Findings 4.3.1 CHD Without Predicted Risk of Hemodynamic Instability at Birth 4.3.2 CHD with Minimal Risk of Hemodynamic Instability During a Transition 4.3.3 CHD with a High Risk of Hemodynamic Instability at Birth 4.4 Role of Maternal Hyperoxygenation Testing 4.5 Mode of Delivery for a Fetus with CHD 4.6 Strategies to Protect the Brain During Transitional Circulation 5 CHD Lesion-Specific Risk Stratification for Delivery Planning 5.1 D-Transposition of the Great Arteries (d-TGA) with Intact Ventricular Septum 5.2 Coarctation of the Aorta 5.3 Hypoplastic Left Heart Syndrome (HLHS) 5.4 Tetralogy of Fallot 5.5 Tetralogy of Fallot with Absent Pulmonary Valve (ToF/APV) 5.6 Severe Ebstein Anomaly or Tricuspid Valve Dysplasia 5.7 Total Anomalous Pulmonary Venous Return (TAPVR) 5.8 Congenital Complete Heart Block 6 Conclusion References Part III: Pediatric Cardiology General Topics 29 History and Physical Examination in Pediatric Cardiology 1 Introduction 2 History 2.1 Neonates and Infants 2.1.1 Exercise Tolerance 2.1.2 Cyanosis 2.1.3 Breathing Pattern 2.1.4 Growth 2.2 Toddlers/Young Children (1-5 Years) 2.3 Children and Adolescents 2.3.1 Exercise Tolerance 2.3.2 Growth and Development 2.3.3 Common Symptoms in Adolescents 2.3.4 Chest Pain 2.3.5 Palpitations 2.3.6 Dizziness/Syncope 2.3.7 Cyanosis 3 Other Aspects of the Cardiovascular-Focused History 3.1 Past Medical/Surgical History 3.2 Family History 3.3 Review of Systems (ROS) 3.4 Social History 3.5 Medications 4 Cardiac Examination 4.1 Initial Steps 4.2 Vital Signs 4.3 General Appearance 4.4 Color 4.5 Pulses 5 Examination of the Peripheral Veins 6 Head and Neck Exam 7 Chest and Lung Exam 8 The Precordium 8.1 Inspection 8.2 Palpation 9 Auscultation 9.1 History 9.2 The Cardiac Cycle 9.3 Equipment and Setting 9.4 Areas of Auscultation 10 Cardiac Sounds 10.1 First Heart Sound 10.2 Second Heart Sound 10.3 Third Heart Sound 10.4 Fourth Heart Sound 10.5 Clicks 10.6 Rubs 10.7 Murmurs 10.7.1 Description 10.7.2 Origin 10.7.3 Pitch 10.7.4 Quality 10.7.5 Radiation 10.7.6 Shape 10.7.7 Timing 10.7.8 Grade 10.7.9 Systolic Murmurs 10.7.10 Diastolic Murmurs 10.7.11 Continuous Murmurs 10.7.12 Combined Systolic/Diastolic Murmurs 10.7.13 Role of Maneuvers 10.7.14 Nonpathologic Murmurs 10.7.15 Still´s Murmur 10.7.16 Pulmonary Flow Murmur 10.7.17 Peripheral Pulmonary Stenosis 10.7.18 Cardiorespiratory ``Murmur´´ 10.7.19 Venous Hum - Supraclavicular Bruit 10.7.20 Bruits 11 Abdomen 12 Musculoskeletal and Skin 13 Neurodevelopment Assessment 14 The History and Physical Examination in the Current Era 14.1 Skills and Training, Point of Care Echo, and Artificial Intelligence 14.2 The Role of Electronic Medical Records (EMR) 14.2.1 Telemedicine/Telehealth 15 Summary References 30 Chest X-Ray in Pediatric Cardiology 1 Introduction 1.1 Usefulness 1.2 Precautions 1.2.1 Radiation 1.2.2 Quality 2 Approach to Chest X-Ray Interpretation 2.1 Normal CXR 2.1.1 Frontal Projection Anteroposterior View Posteroanterior View 2.1.2 Lateral Projection 2.2 Heart Position 2.3 Cardiac Size and Contour 3 Cardiac Pathology and Changes in Cardiac Silhouette 3.1 Cardiac Chambers 3.2 Great Vessels 3.3 Pulmonary Vasculature Changes [4] 4 Congenital Heart Disease and Changes in Cardiac Silhouette 4.1 Atrial Septal Defect (ASD) 4.2 Ventricular Septal Defect (VSD) 4.3 Patent Arterial Duct (PDA) 4.4 Atrioventricular Septal Defects 4.5 Eisenmenger Syndrome 4.6 Pulmonary Stenosis 4.7 Tetralogy of Fallot 4.8 Double-Outlet Right Ventricle 4.9 Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) 4.10 Tricuspid Atresia 4.11 Aortic Stenosis 4.12 Coarctation of Aorta 4.13 Transposition of the Great Arteries (d-TGA) 4.14 Common Arterial Trunk 4.15 Hemitruncus 4.16 Total Anomalous Pulmonary Venous Return (TAPVR) 4.17 Partial Anomalous Pulmonary Venous Return (PAPVR) 4.18 Evaluation of Cardiac Devices References 31 Pediatric Electrocardiography 1 Introduction 2 Technique/Lead Placement 3 Relationship to Cardiac Action Potentials 4 Interpretation 4.1 Rate/Rhythm 4.2 Axis 4.2.1 QRS Axis Deviation 4.3 Intervals (PR, QRS, QT) 4.3.1 PR Interval 4.3.2 QRS Interval 4.3.3 QT Interval 4.4 Voltage 4.4.1 Atrial Abnormality 4.4.2 Ventricular Hypertrophy 4.4.3 Low-Voltage QRS 4.5 ST Segment/T and U Waves 4.6 Qspiepr146 Waves References 32 Pediatric Echocardiography 1 Introduction 2 Ultrasound Physics 2.1 Properties of Ultrasound Waves 2.1.1 Wave Attenuation 2.2 Image Acquisition 2.2.1 Generating Signal 2.2.2 Pulsed Ultrasound 2.3 Techniques Applied in Echocardiography 3 The Complete Pediatric Echocardiogram 3.1 2D Imaging and Anatomic Assessment 3.1.1 Subcostal Imaging Window 3.1.2 Parasternal Imaging Window 3.1.3 Apical Imaging Window 3.1.4 Suprasternal Notch Imaging Window 4 Doppler Imaging 4.1 Foreword Preface Contents About the Editor-in-Chief About the Editors About the Section Editors Managing Editor Contributors Part I: Introduction 1 Pediatric Cardiology: A Journey to the Past 1 Introduction 2 Evolution of Recognizing Cardiovascular Structure and Function 3 The Initial Phases of Pediatric Cardiology 4 Evolution of Electrocardiography 5 Echocardiography: To See Through Sound 6 The History of Medical Literature and Pediatric Cardiology 7 Cardiac Catheterization: The Man Was Daring! 8 Rheumatic Heart Disease: Once the Dominant Lesion in Pediatric Cardiology 9 Kawasaki Disease: Taking Over Rheumatic Heart Disease 10 Training in Pediatric Cardiology 10.1 The Founders 10.2 Governing Bodies 10.3 Changing Training Requirements References 2 Normal Development of the Heart 1 Introduction 2 Initial Formation of the Embryo 3 Looping of the Heart Tube 4 Formation and Separation of the Atrial Chambers 5 Formation and Septation of the Atrioventricular Canal 6 Formation of the Ventricles 7 Formation and Septation of the Aortic and Pulmonary Pathways 8 Development of the Cardiac Conduction System 9 Development of the Coronary Circulation 10 Development During the Fetal and Neonatal Periods References 3 Normal Cardiac Anatomy 1 Introduction 2 Attitudinally Appropriate Descriptions 3 The Cardiac Seat Belt 4 The Cardiac Chambers 4.1 The Morphologically Right Atrium 4.2 The Morphologically Left Atrium 4.3 The Morphologically Right Ventricle 4.4 The Morphologically Left Ventricle 5 The Arterial Roots and Intrapericardial Trunks 6 The Atrioventricular and Arterial Valvar Complexes 6.1 The Atrioventricular Valvar Complexes 6.2 The Arterial Valvar Complexes 7 The Septal Structures 8 The Fibrous Skeleton 9 The Conduction System 10 The Blood Supply to the Heart 10.1 The Coronary Arteries 10.2 The Coronary Veins References 4 Sequential Segmental Analysis of Cardiac Morphology 1 Introduction 2 Sequential Segmental Analysis 3 Arrangement of the Organs 4 Atrial Assessment 5 Determination of the Atrioventricular Connections 6 Types of Atrioventricular Connections 7 Types of Atrioventricular Valves 8 Ventricular Morphology 9 Ventricular Topology 10 Assessment of the Ventriculo-Arterial Junctions 10.1 Infundibular Relationships 10.2 Arterial Relationships References 5 The Role of Genetics in Development of Congenital Heart Disease 1 Introduction 2 Epidemiology and Etiology of CHD 2.1 Incidence 2.2 Prevalence According to Disease Type 3 Etiologies of CHD 3.1 Genetic Factors 3.1.1 Chromosomal Abnormalities 3.1.2 Monogenic Diseases 3.1.3 Copy Number Variation (CNV) 3.2 Environmental Factors 3.3 Multifactorial Inheritance 3.4 Recurrence 3.4.1 Multifactorial Inheritance 3.4.2 Chromosomal and Genetic Abnormalities 3.5 Genetic Counseling 4 Genetic Disorders Associated with CHD 4.1 Down Syndrome 4.1.1 Prevalence 4.1.2 Clinical Features 4.1.3 Heart Disease in Down Syndrome 4.1.4 Genetics 4.2 Trisomy 18 4.2.1 Prevalence 4.2.2 Clinical Features 4.2.3 Heart Disease in Trisomy 18 4.2.4 Genetics 4.3 Trisomy 13 4.3.1 Prevalence 4.3.2 Clinical Features 4.3.3 Heart Disease in Trisomy 13 4.3.4 Genetics 4.3.5 Prognosis and Treatment of Trisomy 18 and 13 4.4 Turner Syndrome 4.4.1 Prevalence 4.4.2 Clinical Features 4.4.3 Heart Disease in Turner Syndrome 4.4.4 Genetics 4.5 22q11.2 Deletion Syndrome 4.5.1 Prevalence 4.5.2 Clinical Features 4.5.3 Heart Disease in 22q11.2 Deletion Syndrome 4.5.4 Genetics 4.6 Williams Syndrome 4.6.1 Prevalence 4.6.2 Clinical Features 4.6.3 Heart Disease in Williams Syndrome 4.6.4 Genetics 4.7 Noonan Syndrome 4.7.1 Prevalence 4.7.2 Clinical Features 4.7.3 Heart Disease in Noonan Syndrome 4.7.4 Genetics 4.8 Marfan Syndrome 4.8.1 Prevalence 4.8.2 Clinical Features 4.8.3 Heart Disease in Marfan Syndrome 4.8.4 Genetics 4.9 CHARGE Syndrome 4.9.1 Prevalence 4.9.2 Clinical Features 4.9.3 Heart Disease in CHARGE Syndrome 4.9.4 Genetics 4.10 Holt-Oram Syndrome 4.10.1 Prevalence 4.10.2 Clinical Features 4.10.3 Heart Disease in Holt-Oram Syndrome 4.10.4 Genetics 4.11 Genetic Abnormalities of Nonsyndromic CHD (Table 4) [81-83] 4.11.1 NKX2.5 4.11.2 GATA4 and GATA6 4.11.3 NOTCH1, NOTCH2, and JAG1 4.11.4 TBX20 5 Recent Advances in the Role of Genetics in CHD 5.1 The Role of Genetics in Heterotaxy Syndrome 5.1.1 Genetic Etiology of Heterotaxy Syndrome 5.2 The Role of Genetics in the Outflow Tract Defects 5.2.1 Identification of a Genetic Etiology of the Outflow Tract Defects 5.2.2 Molecular Mechanisms Underlying the Outflow Tract Development: Interaction Between SHF Cells and CNC Cells 6 Concluding Remarks References 6 Teratogenic Factors Contributing to the Development of Congenital Heart Defects 1 Introduction 2 Understanding Risk Factor Studies 2.1 Understanding Measurements of Associations in Risk Factor Studies of Congenital Heart Defects 2.2 Challenges of Studying Risk Factors for Congenital Heart Defects 2.3 Databases of Patients with Congenital Heart Defects 3 Select Risk Factors 3.1 Maternal Sociodemographic Characteristics 3.1.1 Maternal Age 3.1.2 Maternal Reproductive History 3.1.3 Maternal Stress 3.1.4 Socioeconomic Status 3.2 Maternal Health Conditions 3.2.1 Diabetes Mellitus 3.2.2 Epilepsy 3.2.3 Febrile Illnesses: Upper Respiratory Infection, Genitourinary Infections, and Influenza 3.2.4 Hypercholesterolemia and Atherosclerotic Heart Disease 3.2.5 Hyperhomocysteinemia 3.2.6 Hypertension 3.2.7 Phenylketonuria 3.2.8 Pre-Pregnancy Weight 3.2.9 Maternal Rubella Infection 3.3 Maternal Therapeutic Medications 3.3.1 Antiasthmatics 3.3.2 Antibiotics 3.3.3 Antidepressant Medications 3.3.4 Antiepileptics 3.3.5 Antifungals 3.3.6 Antihypertensives 3.3.7 Anti-Inflammatories 3.3.8 Folic Acid 3.4 Maternal Nontherapeutic Drugs 3.4.1 Alcohol 3.4.2 Cigarette Smoking 3.4.3 Illicit Drugs 3.5 Maternal Environmental Exposures 3.5.1 Air Pollution 3.5.2 Chemical Exposures 3.5.3 Water Contamination 4 Conclusion References 7 Ethical Considerations in Fetal and Pediatric Cardiology 1 Introduction 1.1 Ethics and Pediatric Cardiology 1.2 Ethics, the Discipline 1.3 Ethical Theories and Conceptual Frameworks 1.4 Value Plurality and Culture 2 Ethics in Practice 2.1 Informed Consent 2.2 Child and Adolescent Decision-Making 2.3 Best Interest Standard 2.4 Shared Decision-Making 2.5 Ethical Controversies in Non-intervention 2.6 Ethical Controversies in Intervention 2.7 Extracardiac and Genetic Anomalies 2.8 Pediatric Heart Transplantation 2.8.1 Use of Resources and Organ Allocation 2.8.2 Informed Consent in Transplant 2.8.3 Mechanical Support 2.8.4 Psychosocial Factors 2.8.5 Quality of Life and End-of-Life Care 2.8.6 Regulatory Aspects of Organ Donation and Transplantation 2.9 Use of Ethics Consultation Services 3 Ethics in a Changing World 3.1 Transparency and Regionalization in Pediatric Cardiology and Cardiac Surgery 3.2 Disparities in Care and Social Determinants of Health 3.3 Global Perspective in Congenital Heart Disease 3.4 Innovation in Cardiology and Cardiac Surgery 3.4.1 Innovation Versus Research 3.4.2 New Practices Versus First Practices 4 Conclusion References 8 Quality and Value Improvement in Pediatric Cardiac Care 1 Quality Improvement Background 1.1 Donabedian: Structure, Process, and Outcome 1.2 Institute of Medicine Quality Domains 1.3 Improvement Frameworks: Model for Improvement, Six Sigma, and Lean 1.4 Quality Improvement Tools 1.5 Quality Improvement Versus Research (and Accountability) 2 Safety 2.1 Categorizing Safety 2.2 Measuring Safety 2.3 A Global and Cultural Framework for Safety 2.4 The Future of Safety in Health Care 3 Healthcare Value 3.1 The Formula for Healthcare Value 3.2 The Outcomes Hierarchy 3.3 True Cost 3.4 Healthcare Value and Health Equity 3.5 A Proposed Quality-Value Relationship 4 Variation and Standardization 4.1 Research Studies Characterizing Variation 4.2 Measure Development and Variation Reduction Through Collaboratives 4.3 Improving Quality Through Standardization 5 Learning from Data in QI 5.1 Learning from Data over Time 5.2 Variation and Statistical Quality Improvement Methods 5.3 Run Charts and Statistical Process Control (SPC): A Simple Example 6 External Forces as Drivers of Improvement Efforts 6.1 U.S. News & World Report Best Children ́s Hospital Rankings 6.2 Conquering CHD 7 Local Improvement Structure and Lessons Learned: The Improvement Journey of Two Unique Pediatric Cardiac Programs 8 Conclusions References 9 Research in Pediatric Cardiology 1 Pediatric Cardiology and Clinical Research 2 Quality and Safety in Pediatric Care: Overcoming the Implementation Gap 2.1 Coproduction of Optimal Pediatric Outcomes 2.2 Bringing High Reliability into Pediatric Healthcare 2.3 Enhancing Learning and Improvement Within the Cardiology Microsystem 2.4 A Human Factors-Social-Technical Systems Approach to Healthcare Quality and Patient Safety 2.5 Reducing Diagnostic Errors in Pediatric Cardiology 2.6 Safety and Quality Outcome Measurement and Monitoring 2.7 The Science of Continuous Quality and Performance Improvement 2.8 The Essential Role Teamwork Plays in Enabling Pediatric Safety and Quality 2.9 The Role of Communication and Coordination in Improving Safety and Quality Team Performance 2.10 Turning Healthcare Experts into an Expert Team Requires Substantial Planning and Practice 2.11 The Learning Organization: Capturing Process and Outcomes Failures While Engaging Staff 2.12 Creating a Culture of Trust: The Cornerstone of Clinician and Management Engagement for Safety 2.13 Implementation Science and Approaches to Overcome Unseen Barriers to Change 2.14 Regionalization Learning Collaboratives of Pediatric Cardiac Services 2.15 The Future of Quality Improvement and Culture Change in Pediatrics 3 Future Directions References 10 Fetal Autopsy and Cardiac Registry 1 Introduction 2 Interdepartmental Communication 3 Autopsy Permit 4 The Autopsy 5 Dilation and Evacuation Specimens 6 Cardiac Collections 7 Advancing Modern Collections References 11 Worldwide Practice of Pediatric Cardiology 1 Introduction 2 Challenges in Organizing Cardiac Care in Low- and Low-Middle-Income Countries 2.1 Financial Support 2.2 Access to Care and Poor Awareness 2.3 Education and Training 2.4 Research 2.5 Experience from Local Programs and More Global Evaluations 3 Challenges and Success: Review of Programs 3.1 Mozambique 3.2 Guatemala 3.3 South Africa 3.4 Pakistan 3.5 East Africa 3.6 Lebanon 4 Global Evaluation of Local Programs in LMI Countries 5 Building a Regional Cardiac Program in Low- and Low-Middle-Income Regions: Lessons Learned from International Experts and Pe... 5.1 Twinning Partnership 5.2 A Plan 5.3 Reconnaissance 5.4 Selection of Local Partners 5.5 Continuous Support 5.6 Building a Team Coupled with Continuous Education 5.7 Quality Control 5.8 Equipment 5.9 Local Solutions 5.10 Starting the Program 5.11 Communication with the Community 5.12 Locally Generated Research 6 Conclusion 7 Testimonials 8 Reflections on a Personal Journey to Provide Cardiac Care in Namibia: By Christopher Hugo-Hamman 8.1 Background 8.2 Evolution 8.3 The Culture of Pediatric Cardiac Care 8.4 Human Resources Constraints 8.5 Infrastructure 8.6 Management Systems 8.7 Political Will 9 Testimony of an NGO Director: Steve Sosebee, President of Palestine Children ́s Relief Fund (PCRF) 10 Testimony: Experience of a Pediatric Cardiologist in Rwanda by Emmanuel Rusingiza 10.1 Personal Background 10.2 The State of Pediatric Cardiac Diseases in Public Setting and Access to Care 10.3 Current State of Cardiac Diseases in Rwanda 10.4 Hope for Unmet Ambitions 11 Quotes by Christopher Hugo-Hamman (CHH), Steve Sosebee (StS), Emmanuel Rusingiza (ER), and Daniel De Wolf (DDW) References Part II: Fetal Cardiology 12 Fetal Cardiovascular Physiology 1 Introduction 2 Normal Fetal Circulation 2.1 Course of Blood Flow in the Normal Fetal Circulation 3 The Venous Duct 4 The Oval Foramen 5 The Arterial Duct 5.1 Histology of the Arterial Duct 5.2 Postnatal Ductal Closure 5.3 The Role of the Arterial Duct (AD) in the Presence of CHD 6 Fetal Cardiac Output and Circulatory Regulation 7 The Baroreflex and Chemoreflex in the Fetus 8 The Fetal Myocardium 9 Gas Exchange and O2 Delivery 10 Role of Fetal Hemoglobin 11 Postnatal Circulation 12 Change in Cardiac Output, Hemoglobin, and Myocardium 13 Assessment of Fetal Myocardial Performance References 13 Fetal Echocardiography 1 Background 2 Indications 3 Fetal Echocardiogram 3.1 Cardiac Structure Fig. 10 Standard fetalechoshortaxisviews 3.2 Hemodynamic Assessment Fig. 11 Great vesselshortaxisview. RV right ventricle,Ao aortic valve, PA pulmonary artery 3.2.1 Umbilical Vessels Fig. 12 Umbilical arteryandumbilicalveinDoppler flow 3.2.2 Systemic Veins Fig. 13 Ductus venosus flow. S ventricular systole, D diastole, A atrial systole Fig. 14 Hepatic veinDoppler. S ventricular systole, D diastole, A atrial systole 3.2.3 Pulmonary Veins Fig. 15 Right pulmonaryveinDoppler. S ventricular systole, D diastole, A atrial systole 3.2.4 Atrioventricular Valves Fig. 16 Tricuspidvalveinflow Doppler. E early diastole, A atrial systole 3.2.5 Semilunar Valves 3.2.6 Foramen Ovale Fig. 17 Aortic outflow Doppler.Peakvelocity60cm/s 3.2.7 Aortic Arch 3.2.8 Arterial Ducts 3.3 Rhythm Assessment 3.4 Function Assessment References 14 Fetal Cardiovascular MRI 1 Introduction 2 Rationale for the Development of Fetal Cardiovascular MRI 2.1 Accuracy of Prenatal Diagnosis of CHD 2.2 Longitudinal Monitoring of Fetal Cardiac Treatment and Interventions 2.3 Impairment of Prenatal Brain Development in the CHD Population 2.4 The Role of Placental Disease in CHD Fetuses 3 Safety 4 Technical Aspects of Fetal Cardiovascular MRI 5 Magnetic Resonance Oximetry 6 Improving the Fetal Diagnosis of CHD Using Fetal Cardiovascular MRI 7 Role of Cardiovascular MRI in Understanding Fetal Cardiovascular Physiology 7.1 The Normal Circulation 7.2 Intrauterine Growth Restriction 7.3 Acute Maternal Hyperoxygenation 7.4 Pathophysiology of Congenital Heart Diseases (CHD) 8 Assessment of the Impact of Fetal Interventions by Fetal Cardiovascular MRI 8.1 Invasive Fetal Cardiac Interventions 8.2 Fetal Anemia and Transfusions 8.3 Pharmacological Cardiac Interventions 9 Utility of Fetal Cardiovascular MRI to Investigate the Neurodevelopmental Impact of CHD and IUGR 10 Potential Role of Fetal Cardiovascular MRI for the Assessment of Placental Function 11 How to Perform a Fetal Cardiac MRI: Practical Aspects and Illustrative Cases 12 Current Limitations 13 Future Perspectives 14 Conclusion References 15 Fetal Systemic Venous Anomalies 1 Introduction 2 Superior Caval Vein Anomalies 2.1 Persistent Left Superior Caval Vein to an Intact Coronary Sinus 2.1.1 Morphogenesis 2.1.2 Prevalence and Associated Defects 2.1.3 Fetal Echocardiography 2.1.4 Clinical Implications and Management 2.1.5 Genetics 2.2 Persistent Left Superior Caval Vein to an Unroofed Coronary Sinus 2.2.1 Morphogenesis 2.2.2 Prevalence and Associated Defects 2.2.3 Fetal Echocardiography 2.2.4 Clinical Implications and Management 2.2.5 Genetics 2.3 Left Superior Caval Vein to Left Atrium 2.3.1 Morphogenesis 2.3.2 Prevalence 2.3.3 Fetal Echocardiography 2.3.4 Clinical Implications and Management 2.4 Right SVC to Left Atrium 2.4.1 Morphogenesis 2.4.2 Prevalence 2.4.3 Fetal Echocardiography 2.4.4 Clinical Implications and Management 3 Inferior Caval Vein Anomalies 3.1 Interrupted Inferior Caval Vein with Azygous or Hemiazygous Continuation 3.1.1 Morphogenesis 3.1.2 Prevalence and Associated Defects 3.1.3 Fetal Echocardiography 3.1.4 Clinical Implications and Management 3.2 Bilateral Inferior Caval Veins 3.2.1 Morphogenesis 3.2.2 Prevalence and Associated Defects 3.2.3 Clinical Implications and Management 3.3 Left Inferior Caval Vein to Right Atrium (Absent Right Inferior Caval Vein) 3.3.1 Morphogenesis 3.3.2 Prevalence 3.3.3 Clinical Implications and Management 3.4 Agenesis of the Ductus Venosus 3.4.1 Morphogenesis 3.4.2 Prevalence and Associated Defects 3.4.3 Fetal Echocardiography 3.4.4 Clinical Implications and Management 3.4.5 Genetics References 16 Fetal Pulmonary Venous Anomalies 1 General Introduction 2 Total Anomalous Pulmonary Venous Return (TAPVR) 2.1 Introduction: Prevalence, Embryology, and Associated Lesions 2.2 Prenatal Ultrasound Manifestations 2.3 Prenatal Ultrasound Findings in TAPVR Types 2.3.1 Supracardiac TAPVR 2.3.2 Infracardiac TAPVR 2.3.3 Cardiac TAPVR 2.3.4 Mixed Type Prenatal Follow-Up and Delivery Preparation 2.4 Prenatal Counseling 3 Partial Anomalous Pulmonary Venous Return (PAPVR) 3.1 Introduction: Prevalence, Embryology, and Associated Lesions 3.2 Prenatal Ultrasound Manifestations 3.3 Prenatal Counseling References 17 Fetal Ebstein Malformation 1 Introduction 2 Morphology and Associated Anomalies 3 Associated Anomalies 4 Manifestation on Obstetrical Ultrasound 5 Antenatal History and Role of Genetics 6 Fetal Echocardiography 7 Fetal Life Follow-Up, What to Expect 8 Interventions 9 Hemodynamic Changes at Birth 10 Preparation at Delivery and Immediate Postnatal Care 10.1 Cardiac Function 10.2 Tricuspid Insufficiency 10.3 Pulmonary Flow 10.4 Presence of Arrhythmia 10.5 Lung Hypoplasia or Presence of Other Malformations 10.6 Presence of Hydrops 10.7 Neonatal Surgical Intervention References 18 Fetal Pulmonary Stenosis 1 Introduction 2 Classification 3 Associated Cardiac Findings in Pulmonary Stenosis 3.1 Poststenotic Dilatation of the Main Pulmonary Artery 3.2 Nondilated Main Pulmonary Artery in Pulmonary Stenosis 3.3 Tricuspid Valve Morphology, Size, and Function 3.4 Right Ventricular Morphology and Function 3.5 Right Atrial Dilatation 3.6 Reversal of Flow in the Arterial Duct 3.7 Ventriculocoronary Arterial Communication 4 Natural History 5 Fetal and Neonatal Management References 19 Fetal Left Heart Obstructive Lesions: Aortic Stenosis, Shone ́s Complex, and Aortic Arch Obstruction 1 Aortic Valve Stenosis 1.1 Introduction 1.2 Manifestation of Lesion in OB Ultrasound 1.3 History Taking 1.4 Details of Fetal Echocardiography 1.5 Fetal Life Follow-Up 1.6 Interventions 1.7 Expected Changes in Hemodynamics at Birth 1.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 1.9 Parental Counseling 2 Coarctation of the Aorta 2.1 Introduction 2.2 Manifestations of Lesion in OB Ultrasound 2.3 History Taking and Additional Testing 2.4 Details of Fetal Echocardiography 2.5 Fetal Life Follow-Up 2.6 Interventions 2.7 Expected Changes in Hemodynamics at Birth 2.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 2.9 Parental Counseling 3 Shone ́s Complex 3.1 Introduction 3.2 Manifestations of Lesion in OB Ultrasound 3.3 History Taking 3.4 Details of Fetal Echocardiography 3.5 Fetal Life Follow-Up 3.6 Interventions 3.7 Expected Changes in Hemodynamics at Birth 3.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 3.9 Parental Counseling 4 Interrupted Aortic Arch 4.1 Introduction 4.2 Manifestations of Lesion in OB Ultrasound 4.3 History Taking 4.4 Details of Fetal Echocardiography 4.5 Fetal Life Follow-Up 4.6 Interventions 4.7 Expected Changes in Hemodynamics at Birth 4.8 Preparation at Delivery and Immediate Postnatal Course/Delivery Room Planning 4.9 Parental Counseling References 20 Fetal Hypoplastic Left Heart Syndrome 1 Introduction 2 Cardiac Anatomy in Hypoplastic Left Heart Syndrome and Anatomic Associations 3 Incidence and Genetics 4 Prenatal Detection of HLHS 5 Evolving HLHS: Possible Mechanisms and Prenatal Findings 6 Cardiac Structure and Fetal Echocardiographic Features in HLHS 6.1 Systemic Venous Return and Right Atrium 6.2 Atrial Septum and Oval Foramen 7 Pulmonary Veins 8 Levo-atrial Cardinal Vein 9 Mitral Valve 9.1 Left Ventricle 9.2 Aortic Valve 10 Aortic Arch 11 Evaluation of the RV 12 Tricuspid Valve Morphology and Function 13 Cerebral Blood Flow Pattern in HLHS Fetus 14 Impact of Prenatal Diagnosis of HLHS 15 Delivery Planning for a Fetus with HLHS 16 Parental Counseling References 21 Fetal Transposition of the Great Arteries 1 Transposition 1.1 Introduction and Prevalence 1.2 Morphology 1.2.1 Prenatal Genetic Testing 1.3 Pathophysiological Observations in TGA During Prenatal Life 1.4 Prenatal Maternal Hyperoxia 1.5 Echocardiographic Findings 1.6 Transabdominal Imaging 1.7 Associated Defects 1.8 Differential Diagnosis 1.9 Fetal Life Follow-Up, What to Expect 1.10 Hemodynamic Changes at Birth and Clinical Presentation 1.11 Outcome and Prognosis 2 Corrected Transposition 2.1 Introduction and Prevalence 2.2 Associated Defects 2.3 Manifestation in Obstetrical Ultrasound 2.4 Prenatal Genetic Testing 2.5 Fetal Echocardiography 2.6 Fetal Life Follow-Up, What to Expect 2.7 Outcome and Prognosis 2.8 Postnatal Management 3 Conclusion References 22 Fetal Conotruncal Defects 1 Introduction 1.1 Genetics of Conotruncal Defects 2 Tetralogy of Fallot 2.1 Introduction 2.2 Anatomy and Physiology 2.3 Fetal Diagnosis 2.4 Fetal Echocardiography 2.5 Special Fetal Imaging Considerations 2.6 Outcomes 2.7 Fetal Follow-Up and Counseling 2.8 Delivery Planning 3 Common Arterial Trunk 3.1 Anatomy and Physiology 3.2 Fetal Diagnosis 3.3 Fetal Echocardiography 3.4 Fetal Follow-Up and Counseling 3.5 Delivery Planning 4 Double-Outlet Right Ventricle 4.1 Anatomy and Physiology 4.2 Fetal Diagnosis 4.3 Fetal Echocardiography 4.4 Outcomes 4.5 Fetal Follow-Up and Counseling 4.6 Delivery Planning References 23 Fetal Functionally Single Ventricle 1 Pulmonary Atresia with Intact Ventricular Septum 1.1 Introduction 1.2 Manifestation in Obstetrical Ultrasound and History Taking 1.3 Details of Fetal Echocardiography 1.4 Fetal Follow-Up 1.5 Fetal Interventions 1.6 Delivery Preparation and Postnatal Outcome 2 Unbalanced Atrioventricular Canal 2.1 Introduction 2.2 Manifestation in Obstetrical Ultrasound and History Taking 2.3 Details of Fetal Echocardiography 2.4 Fetal Follow-Up 2.5 Delivery Preparation and Postnatal Outcome 3 Tricuspid Atresia 3.1 Introduction 3.2 Manifestation in Obstetrical Ultrasound and History Taking 3.3 Details of Fetal Echocardiography 3.4 Fetal Follow-Up 3.5 Delivery Preparation and Postnatal Outcome 4 Double-Inlet Left Ventricle 4.1 Introduction 4.2 Manifestation in Obstetrical Ultrasound and History Taking 4.3 Details of Fetal Echocardiography 4.4 Fetal Follow-Up 4.5 Delivery Preparation and Postnatal Outcome References 24 Fetal Vascular Ring 1 Introduction 1.1 Normal Arch Embryology and Identification in Fetal Echocardiography 2 Right-Sided Aortic Arches and Subtypes 2.1 Right Aortic Arch with Mirror Image Branching 2.2 Right Aortic Arch with an Aberrant Left Subclavian Artery 2.3 Other Right Aortic Arch Variants 3 Double Aortic Arch 4 Alternative Imaging for RAA and DAA 5 Left Pulmonary Artery (LPA Sling) 6 Prenatal Counseling References 25 Fetal Hydrops and Heart Failure 1 Introduction 2 Pathophysiology 3 Etiology 3.1 Structural Cardiac Abnormalities 3.2 Cardiac Failure 3.3 Arrhythmias 3.4 High-Output Failure 3.5 Idiopathic Arterial Calcification 3.6 Genetic Etiologies 3.7 Twin-Twin Transfusion and TRAP Sequence 4 Diagnostic Evaluation 5 Evaluation by Fetal Echocardiogram 6 Fetal Interventions 7 Preparation for Delivery and Immediate Postnatal Care 8 Outcomes References 26 Fetal Arrhythmia: Bradycardia and Tachycardia 1 Introduction 2 Echocardiographic Assessment of Fetal Rhythm 2.1 2D Assessment of Fetal Heart Rhythm 2.2 M-Mode Assessment of Fetal Heart Rhythm 2.3 Spectral Doppler Assessment of Fetal Heart Rhythm 2.4 Tissue Doppler Assessment of Fetal Heart Rhythm 2.5 Fetal Magnetocardiography Assessment of the Fetal Heart Rhythm 3 Types of Arrhythmias 3.1 Irregular Heart Rhythm 3.1.1 Premature Atrial Contractions 3.1.2 Premature Ventricular Contractions 3.1.3 Second-Degree AV Block 3.2 Tachyarrhythmias 3.2.1 Sinus Tachycardia 3.2.2 Reentrant Supraventricular Tachycardia 3.2.3 Treatment of SVT 3.2.4 Treatment of SVT in the Hydropic Fetus 3.2.5 Atrial Flutter 3.2.6 Treatment of Atrial Flutter 3.2.7 Ventricular Tachycardia 3.2.8 Rare Fetal Tachyarrhythmias 3.3 Bradyarrhythmia 3.3.1 Sinus Bradycardia 3.3.2 Complete Heart Block 3.3.3 Treatment of Complete Heart Block 3.3.4 Blocked Atrial Bigeminy 4 Channelopathies 5 Conclusions References 27 Fetal Cardiac Intervention 1 Introduction 2 Severe Mid-gestation Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome 2.1 Patient Selection 2.2 Technical Aspects and Procedural Success 2.3 Postnatal Outcome 3 Pulmonary Atresia with Intact Ventricular Septum or Critical Pulmonary Stenosis and Evolving Hypoplastic Right Heart Syndrome 3.1 Patient Selection 3.2 Technical Aspects and Procedural Success 3.3 Postnatal Outcome 4 Established Hypoplastic Left Heart Syndrome with Intact or Highly Restrictive Atrial Septum 4.1 Patient Selection 4.2 Technical Aspects and Procedural Success 4.3 Postnatal Outcome 5 Less Commonly Performed Fetal Cardiac Interventions 6 Future Directions References 28 Delivery Planning for Complex Congenital Heart Disease 1 Introduction 2 Fetal Circulation and Transitional Physiology 3 In Utero Progression of CHD 4 Principles for Delivery Planning 4.1 A Multidisciplinary Approach for Delivery Planning 4.2 Choosing Appropriate Delivery Location Based on ``Levels of Neonatal Care ́ ́ 4.3 Classification of CHD Severity Based on Fetal Echocardiographic Findings 4.3.1 CHD Without Predicted Risk of Hemodynamic Instability at Birth 4.3.2 CHD with Minimal Risk of Hemodynamic Instability During a Transition 4.3.3 CHD with a High Risk of Hemodynamic Instability at Birth 4.4 Role of Maternal Hyperoxygenation Testing 4.5 Mode of Delivery for a Fetus with CHD 4.6 Strategies to Protect the Brain During Transitional Circulation 5 CHD Lesion-Specific Risk Stratification for Delivery Planning 5.1 D-Transposition of the Great Arteries (d-TGA) with Intact Ventricular Septum 5.2 Coarctation of the Aorta 5.3 Hypoplastic Left Heart Syndrome (HLHS) 5.4 Tetralogy of Fallot 5.5 Tetralogy of Fallot with Absent Pulmonary Valve (ToF/APV) 5.6 Severe Ebstein Anomaly or Tricuspid Valve Dysplasia 5.7 Total Anomalous Pulmonary Venous Return (TAPVR) 5.8 Congenital Complete Heart Block 6 Conclusion References Part III: Pediatric Cardiology General Topics 29 History and Physical Examination in Pediatric Cardiology 1 Introduction 2 History 2.1 Neonates and Infants 2.1.1 Exercise Tolerance 2.1.2 Cyanosis 2.1.3 Breathing Pattern 2.1.4 Growth 2.2 Toddlers/Young Children (1-5 Years) 2.3 Children and Adolescents 2.3.1 Exercise Tolerance 2.3.2 Growth and Development 2.3.3 Common Symptoms in Adolescents 2.3.4 Chest Pain 2.3.5 Palpitations 2.3.6 Dizziness/Syncope 2.3.7 Cyanosis 3 Other Aspects of the Cardiovascular-Focused History 3.1 Past Medical/Surgical History 3.2 Family History 3.3 Review of Systems (ROS) 3.4 Social History 3.5 Medications 4 Cardiac Examination 4.1 Initial Steps 4.2 Vital Signs 4.3 General Appearance 4.4 Color 4.5 Pulses 5 Examination of the Peripheral Veins 6 Head and Neck Exam 7 Chest and Lung Exam 8 The Precordium 8.1 Inspection 8.2 Palpation 9 Auscultation 9.1 History 9.2 The Cardiac Cycle 9.3 Equipment and Setting 9.4 Areas of Auscultation 10 Cardiac Sounds 10.1 First Heart Sound 10.2 Second Heart Sound 10.3 Third Heart Sound 10.4 Fourth Heart Sound 10.5 Clicks 10.6 Rubs 10.7 Murmurs 10.7.1 Description 10.7.2 Origin 10.7.3 Pitch 10.7.4 Quality 10.7.5 Radiation 10.7.6 Shape 10.7.7 Timing 10.7.8 Grade 10.7.9 Systolic Murmurs 10.7.10 Diastolic Murmurs 10.7.11 Continuous Murmurs 10.7.12 Combined Systolic/Diastolic Murmurs 10.7.13 Role of Maneuvers 10.7.14 Nonpathologic Murmurs 10.7.15 Still ́s Murmur 10.7.16 Pulmonary Flow Murmur 10.7.17 Peripheral Pulmonary Stenosis 10.7.18 Cardiorespiratory ``Murmur ́ ́ 10.7.19 Venous Hum - Supraclavicular Bruit 10.7.20 Bruits 11 Abdomen 12 Musculoskeletal and Skin 13 Neurodevelopment Assessment 14 The History and Physical Examination in the Current Era 14.1 Skills and Training, Point of Care Echo, and Artificial Intelligence 14.2 The Role of Electronic Medical Records (EMR) 14.2.1 Telemedicine/Telehealth 15 Summary References 30 Chest X-Ray in Pediatric Cardiology 1 Introduction 1.1 Usefulness 1.2 Precautions 1.2.1 Radiation 1.2.2 Quality 2 Approach to Chest X-Ray Interpretation 2.1 Normal CXR 2.1.1 Frontal Projection Anteroposterior View Posteroanterior View 2.1.2 Lateral Projection 2.2 Heart Position 2.3 Cardiac Size and Contour 3 Cardiac Pathology and Changes in Cardiac Silhouette 3.1 Cardiac Chambers 3.2 Great Vessels 3.3 Pulmonary Vasculature Changes [4] 4 Congenital Heart Disease and Changes in Cardiac Silhouette 4.1 Atrial Septal Defect (ASD) 4.2 Ventricular Septal Defect (VSD) 4.3 Patent Arterial Duct (PDA) 4.4 Atrioventricular Septal Defects 4.5 Eisenmenger Syndrome 4.6 Pulmonary Stenosis 4.7 Tetralogy of Fallot 4.8 Double-Outlet Right Ventricle 4.9 Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) 4.10 Tricuspid Atresia 4.11 Aortic Stenosis 4.12 Coarctation of Aorta 4.13 Transposition of the Great Arteries (d-TGA) 4.14 Common Arterial Trunk 4.15 Hemitruncus 4.16 Total Anomalous Pulmonary Venous Return (TAPVR) 4.17 Partial Anomalous Pulmonary Venous Return (PAPVR) 4.18 Evaluation of Cardiac Devices References 31 Pediatric Electrocardiography 1 Introduction 2 Technique/Lead Placement 3 Relationship to Cardiac Action Potentials 4 Interpretation 4.1 Rate/Rhythm 4.2 Axis 4.2.1 QRS Axis Deviation 4.3 Intervals (PR, QRS, QT) 4.3.1 PR Interval 4.3.2 QRS Interval 4.3.3 QT Interval 4.4 Voltage 4.4.1 Atrial Abnormality 4.4.2 Ventricular Hypertrophy 4.4.3 Low-Voltage QRS 4.5 ST Segment/T and U Waves 4.6 Qspiepr146 Waves References 32 Pediatric Echocardiography 1 Introduction 2 Ultrasound Physics 2.1 Properties of Ultrasound Waves 2.1.1 Wave Attenuation 2.2 Image Acquisition 2.2.1 Generating Signal 2.2.2 Pulsed Ultrasound 2.3 Techniques Applied in Echocardiography 3 The Complete Pediatric Echocardiogram 3.1 2D Imaging and Anatomic Assessment 3.1.1 Subcostal Imaging Window 3.1.2 Parasternal Imaging Window 3.1.3 Apical Imaging Window 3.1.4 Suprasternal Notch Imaging Window 4 Doppler Ima