BOOK
Hemodynamics and Cardiology: Neonatology Questions and Controversies E-Book
Charles S. Kleinman | Istvan Seri
(2012)
Additional Information
Book Details
Abstract
Hemodynamics and Cardiology, a volume in Dr. Polin’s Neonatology: Questions and Controversies Series, offers expert authority on the toughest cardiovascular challenges you face in your practice. This medical reference book will help you provide better evidence-based care and improve patient outcomes with research on the latest advances.
- Reconsider how you handle difficult practice issues with coverage that addresses these topics head on and offers opinions from the leading experts in the field, supported by evidence whenever possible.
- Find information quickly and easily with a consistent chapter organization.
- Get the most authoritative advice available from world-class neonatologists who have the inside track on new trends and developments in neonatal care.
- Stay current in practice with coverage on issues such as the clinical implications of near-infrared spectroscopy in neonates, MRI imaging and neonatal hemodynamics, and hybrid management techniques for congenital heart disease.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front cover | cover | ||
| Pin page | ifc_i | ||
| Half title page | i | ||
| Front Matter | ii | ||
| Hemodynamics and Cardiology: Neonatology Questions and Controversies | iii | ||
| Copyright page | iv | ||
| Dedication | v | ||
| Contributors | vii | ||
| Series Foreword | xv | ||
| Preface | xvii | ||
| Table of Contents | xix | ||
| Part I Neonatal Hemodynamics | 1 | ||
| Section A Principles of Developmental Cardiovascular Physiology and Pathophysiology | 1 | ||
| Chapter 1 Principles of Developmental Cardiovascular Physiology and Pathophysiology | 3 | ||
| Principles of Developmental Physiology | 3 | ||
| Fetal Circulation | 3 | ||
| Transitional Physiology | 4 | ||
| Postnatal Circulation | 4 | ||
| Pressure, Flow, and Resistance | 4 | ||
| Organ Blood Flow Distribution | 5 | ||
| Microcirculatory Physiology | 5 | ||
| Myocardial Function—Developmental Aspects | 6 | ||
| Impact of the Immature Autonomic Nervous System on Regulating Cardiac Function and Vascular Tone | 6 | ||
| Developmental Cardiovascular Pathophysiology: Etiology and Pathophysiology of Neonatal Shock | 7 | ||
| Definition and Phases of Shock | 8 | ||
| Etiology of Neonatal Shock | 10 | ||
| Hypovolemia | 10 | ||
| Myocardial Dysfunction | 11 | ||
| Vasodilation | 13 | ||
| Case Study | 16 | ||
| Adrenal Insufficiency (See Chapters 12 and 14) | 19 | ||
| Downregulation of Adrenergic Receptors | 20 | ||
| References | 23 | ||
| Chapter 2 Autoregulation of Vital and Nonvital Organ Blood Flow in the Preterm and Term Neonate | 29 | ||
| Regulation of Arterial Tone | 31 | ||
| The Role of Conduit Arteries in Regulating Vascular Resistance | 31 | ||
| Arterial Reaction to Pressure (Autoregulation) | 31 | ||
| Interaction of Autoregulation and Hypoxic Vasodilatation | 32 | ||
| Interaction of Autoregulation and Pco2 | 33 | ||
| Interaction of Autoregulation and Functional Activation (Metabolic Blood Flow Control) | 34 | ||
| Flow-mediated Vasodilatation | 34 | ||
| Sympathetic Nervous System | 34 | ||
| Humoral Factors in General Circulation | 35 | ||
| Summary | 35 | ||
| Blood Flow to the Brain | 35 | ||
| Autoregulation of Cerebral Blood Flow in the Immature Brain | 35 | ||
| Effect of Carbon Dioxide on Cerebral Blood Flow | 37 | ||
| Metabolic Control of Blood Flow to the Brain | 37 | ||
| Adrenergic Mechanisms Affecting Cerebral Blood Flow | 38 | ||
| Effect of Medications on Cerebral Blood Flow | 38 | ||
| Ischemic Thresholds in the Brain | 38 | ||
| Blood Flow to Other Organs | 39 | ||
| Kidney | 39 | ||
| Liver | 39 | ||
| Stomach and Intestines | 40 | ||
| Distribution of Cardiac Output in the Healthy Human Neonate | 40 | ||
| Blood Flow to the Upper Part of the Body | 40 | ||
| Blood Flow to the Lower Part of the Body | 41 | ||
| Mechanisms Governing the Redistribution of Cardiac Output in the Fetal ‘“Dive” Reflex | 42 | ||
| Aerobic Diving | 42 | ||
| Reactions to Hypoxia | 42 | ||
| Modifying Effects | 42 | ||
| Distribution of Cardiac Output in the Shocked Newborn | 42 | ||
| The Term Neonate with Low Cardiac Output | 42 | ||
| The Very Preterm Neonate During Immediate Postnatal Adaptation | 43 | ||
| Other Scenarios | 43 | ||
| Conclusion | 43 | ||
| References | 43 | ||
| Chapter 3 Definition of Normal Blood Pressure Range: | 49 | ||
| Case Study | 49 | ||
| Measuring Blood Pressure | 50 | ||
| Direct Measurement of Blood Pressure | 50 | ||
| Noninvasive Measurement of Blood Pressure | 52 | ||
| Normative Data for Blood Pressure in Neonates | 56 | ||
| Adjuncts to Blood Pressure Measurement in the Diagnosis of Compromised Circulatory Function | 63 | ||
| Urine Output | 64 | ||
| Metabolic Acidosis | 64 | ||
| Hyperkalemia | 64 | ||
| Heart Rate | 65 | ||
| Capillary Refill Time and Central-Peripheral Temperature Difference | 65 | ||
| Clinical Factors that May Affect Blood Pressure | 66 | ||
| Maternal Age and Blood Pressure | 66 | ||
| Route of Delivery | 67 | ||
| Time of Umbilical Cord Clamping | 67 | ||
| Patent Ductus Arteriosus | 67 | ||
| Apnea | 68 | ||
| Respiratory Support | 68 | ||
| Antenatal Steroids | 69 | ||
| Therapeutic Hypothermia | 70 | ||
| Other Indicators of Changes in Circulatory Function | 71 | ||
| Conclusion | 71 | ||
| References | 72 | ||
| Section B Diagnosis of Neonatal Shock: Methods and Their Clinical Applications | 79 | ||
| Chapter 4 Methods to Assess Systemic and Organ Blood Flow in the Neonate | 81 | ||
| Doppler Ultrasound | 82 | ||
| Doppler Principle | 82 | ||
| First Instruments | 83 | ||
| Indices of Pulsatility | 83 | ||
| Blood Flow Velocity | 84 | ||
| Volumetric Measurements | 84 | ||
| Near-Infrared Spectroscopy | 84 | ||
| Geometry | 84 | ||
| Algorithms and Wavelength | 85 | ||
| Pathlength | 85 | ||
| Quantification of Cerebral Blood Flow | 85 | ||
| Assumptions | 85 | ||
| Reproducibility and Validation | 85 | ||
| Indocyanine Green as an Alternative Tracer | 85 | ||
| Trend Monitoring of Hemoglobin Signals | 86 | ||
| The Hemoglobin Difference, or Oxygenation Index | 87 | ||
| Coherence of the Spontaneous Variability of Cerebral Hemoglobin Oxygenation and Variability of Arterial Blood Pressure as an Indication of Cerebral Autoregulation | 87 | ||
| Diffuse Correlation Spectroscopy | 87 | ||
| Quantification of Hemoglobin-Oxygen Saturation | 87 | ||
| Bias of Tissue Oxygen Saturation | 88 | ||
| Precision of the Tissue Oxygen Saturation | 88 | ||
| Importance of the Low Precision of the Tissue Oxygen Saturation | 88 | ||
| Magnetic Resonance Imaging | 88 | ||
| Measurement of Blood Flow | 88 | ||
| Qualitative Flow Imaging (Fig. 4-3) | 89 | ||
| Quantitative Flow Imaging | 89 | ||
| Kety-Schmidt Method | 90 | ||
| 133Xe Clearance | 90 | ||
| Single Photon Emission Computed Tomography | 91 | ||
| Stable Xenon-Enhanced Computed Tomography | 91 | ||
| Positron Emission Tomography | 91 | ||
| Other Methods | 92 | ||
| Measurement of Flow to Other Organs | 92 | ||
| Conclusion | 92 | ||
| References | 92 | ||
| Chapter 5 Functional Echocardiography in the Neonatal Intensive Care Unit | 95 | ||
| Doppler Ultrasound | 96 | ||
| Two-Dimensional Imaging and Normal Cardiac Structure | 96 | ||
| Types of Doppler | 97 | ||
| What Can Be Measured with Functional Echocardiography in the Neonatal Intensive Care Units? | 98 | ||
| Ductal Shunting | 100 | ||
| Ductal Patency and Direction of Shunting | 100 | ||
| Determination of Hemodynamic Significance | 102 | ||
| Natural History of Postnatal Constriction | 103 | ||
| Atrial Shunting | 104 | ||
| Pulmonary Artery Pressure | 105 | ||
| Pulmonary Artery Pressure from a Ductal Shunt | 106 | ||
| Pulmonary Artery Pressure from Tricuspid Incompetence | 107 | ||
| Pulmonary Artery Doppler Time to Peak Velocity | 108 | ||
| Measurement of Blood Flow and Cardiac Output | 109 | ||
| Left Ventricular Output | 109 | ||
| Right Ventricular Output | 111 | ||
| Superior Vena Cava Flow | 112 | ||
| Left Pulmonary Artery Velocities: Pulmonary Blood Flow | 112 | ||
| Myocardial Function Measures | 114 | ||
| Myocardial Function: Future Developments | 116 | ||
| Diastolic Function | 116 | ||
| Tissue Doppler | 116 | ||
| Functional Echocardiography in the Neonatal Intensive Care Units in Specific Clinical Situations | 118 | ||
| Very Preterm Baby During the Transitional Period | 119 | ||
| Measurement of Systemic Blood Flow | 119 | ||
| Assessment of Early Ductal Constriction and Shunt Direction | 119 | ||
| Preterm Infant with Suspected PDA | 120 | ||
| Baby with Clinically Suspected Circulatory Compromise | 120 | ||
| Baby with Suspected Primary Persistent Pulmonary Hypertension of the Newborn | 121 | ||
| Conclusion | 121 | ||
| References | 121 | ||
| Chapter 6 Assessment of Cardiac Output in Neonates: | 125 | ||
| Fick Principle | 126 | ||
| Oxygen Fick (O2-Fick) Method | 127 | ||
| Pulmonary Oxygen Uptake (VO2) | 127 | ||
| Oxygen Concentration Gradient (CaO2 − CvO2) | 128 | ||
| Cardiac Index (Calculation Examples) | 129 | ||
| Carbon Dioxide Fick (CO2-Fick) Method | 130 | ||
| Modified Carbon Dioxide Fick Method (mCO2F) | 130 | ||
| Carbon Dioxide Rebreathing Technology (CO2R) | 131 | ||
| Echocardiography | 131 | ||
| Electrical Cardiometry | 134 | ||
| Obtaining the Impedance Cardiogram | 134 | ||
| Measurement of Thoracic Electrical Bio-impedance | 135 | ||
| Bio-impedance and Bio-reactance | 136 | ||
| Interpretation of the Impedance Cardiogram | 139 | ||
| Impedance Cardiography/Impedance Plethysmography | 140 | ||
| Alignment of Erythrocytes and Electrical Velocimetry | 141 | ||
| Pulse Contour Method | 143 | ||
| PiCCO Technology | 145 | ||
| LiDCO Technology | 146 | ||
| FloTrac System | 146 | ||
| Conclusion | 146 | ||
| References | 148 | ||
| Chapter 7 Near-Infrared Spectroscopy and Its Use for the Assessment of Tissue Perfusion in the Neonate | 151 | ||
| Principles of Near-Infrared Spectroscopy | 152 | ||
| Near-Infrared Spectrophotometers | 152 | ||
| Continuous Wave Instruments | 153 | ||
| Spatially Resolved Spectroscopy | 155 | ||
| Time-of-flight Instruments | 156 | ||
| Frequency Domain Instruments | 156 | ||
| Measurements of Physiologic Variables | 156 | ||
| Venous Oxygen Saturation | 157 | ||
| Cerebral Venous Oxygen Saturation | 157 | ||
| Peripheral Venous Oxygen Saturation | 158 | ||
| Blood Flow | 160 | ||
| Physiologic Observations Using Near-Infrared Spectroscopy | 161 | ||
| Oxygen Delivery | 161 | ||
| Factors Determining Oxygen Delivery | 162 | ||
| Effect of Anemia | 162 | ||
| Cerebral Oxygen Delivery | 162 | ||
| Cerebral Blood Flow (CBF) | 162 | ||
| Peripheral Blood Flow | 163 | ||
| Oxygen Consumption | 164 | ||
| Cerebral Venous Oxygen Saturation and Consumption | 165 | ||
| Peripheral Venous Oxygen Saturation and Consumption | 165 | ||
| Fractional Oxygen Extraction | 165 | ||
| Oxygen Delivery–Consumption Coupling | 168 | ||
| Early Postnatal Adaptation | 169 | ||
| References | 170 | ||
| Chapter 8 Clinical Applications of Near-Infrared Spectroscopy in Neonates | 173 | ||
| Feasibility of Near-Infrared Spectroscopy-Monitored rScO2 and cFTOE in Clinical Practice in the NICU | 174 | ||
| Clinical Applications | 176 | ||
| Application of the Sensor and Its Pitfalls | 176 | ||
| Relation to Other Monitoring Devices | 177 | ||
| Clinical Conditions Associated with Low rScO2 | 177 | ||
| Clinical Conditions Associated with High rScO2 Values | 182 | ||
| Conclusion | 183 | ||
| References | 183 | ||
| Chapter 9 Advanced Magnetic Resonance Neuroimaging Techniques in the Neonate with a Focus on Hemodynamic-Related Brain Injury | 187 | ||
| Magnetic Resonance–Compatible Neonatal Incubator | 187 | ||
| Magnetic Resonance–Compatible Video Monitoring System | 188 | ||
| Neonatal-Sized Head Coil | 188 | ||
| Diffusion-Weighted Imaging and Diffusion Tensor Imaging of the Neonatal Brain | 188 | ||
| Perfusion Imaging of the Neonatal Brain | 190 | ||
| Exogenous Contrast Agents | 191 | ||
| Blood Oxygen Level–Dependent Functional Magnetic Resonance Imaging | 191 | ||
| Arterial Spin Labeling | 194 | ||
| Continuous Arterial Spin Labeling | 194 | ||
| Pulsed Arterial Spin Labeling | 194 | ||
| Quantitative Proton Magnetic Resonance Spectroscopy of the Neonatal Brain | 195 | ||
| Acknowledgments | 196 | ||
| References | 197 | ||
| Chapter 10 Cardiovascular Magnetic Resonance in the Study of Neonatal Hemodynamics | 199 | ||
| Current Understanding of Neonatal Hemodynamics | 200 | ||
| Current Cotside Circulatory Assessment | 200 | ||
| Optimal Circulatory Management | 200 | ||
| Current Cardiovascular Magnetic Resonance Imaging | 201 | ||
| Success of Cardiovascular Magnetic Resonance in the Adult Population | 202 | ||
| Performing Cardiovascular Magnetic Resonance Imaging in the Newborn | 202 | ||
| Adapting Cardiovascular Magnetic Resonance for Use in Newborns | 203 | ||
| Cine Cardiovascular Magnetic Resonance | 203 | ||
| Phase Contrast Cardiovascular Magnetic Resonance | 204 | ||
| Emerging Cardiac Magnetic Resonance Imaging | 206 | ||
| 3-D Visualization of Flow with Phase Contrast | 206 | ||
| Assessment of Myocardial Motion | 207 | ||
| Potential Role of Cardiovascular Magnetic Resonance in the Study of Neonatal Hemodynamics | 208 | ||
| Definition of the Pathophysiology of Circulatory Failure | 208 | ||
| Acting as a Biomarker in Studies Assessing the Cardiovascular Effects of Vasopressor/Inotropes, Inotropes, and Lusitropes | 208 | ||
| Guiding the Development of Emerging Echocardiographic Techniques | 209 | ||
| Advantages and Disadvantages of Functional Cardiovascular Magnetic Resonance Imaging | 209 | ||
| Conclusion | 210 | ||
| Acknowledgments | 210 | ||
| References | 210 | ||
| Chapter 11 Assessment of the Microcirculation in the Neonate | 215 | ||
| Why Assess the Microcirculation? | 215 | ||
| Where to Study the Microcirculation in the Human Newborn? | 216 | ||
| Laser Doppler Imaging | 217 | ||
| Laser Doppler Flowmetry | 218 | ||
| Assessment of Skin Microcirculation Responses | 218 | ||
| Postocclusive Reactive Hyperemia | 218 | ||
| Local Thermal Hyperemia | 220 | ||
| Iontophoresis | 221 | ||
| Microvasculature of the Preterm Neonate Studied by LDF | 221 | ||
| Gestation Differences | 222 | ||
| Peripheral Microvascular Blood Flow and Neonatal Gender | 224 | ||
| Mechanisms of Preterm Microvascular Control | 225 | ||
| Vasodilators Regulating Microvascular Tone | 225 | ||
| Nitric Oxide | 226 | ||
| Carbon Monoxide | 226 | ||
| Hydrogen Sulfide | 226 | ||
| Vasoconstrictors Regulating Microvascular Tone | 228 | ||
| Videomicroscopy | 229 | ||
| Retinography and Cardiovascular Programming | 229 | ||
| Future Applications in Neonatal Medicine | 230 | ||
| Conclusion | 230 | ||
| References | 230 | ||
| Section C Clinical Presentations and Relevance of Neonatal Shock | 235 | ||
| Chapter 12 Clinical Presentations of Neonatal Shock: | 237 | ||
| Definition of Hypotension and its Relationship to Low Systemic Perfusion | 238 | ||
| The Transitional Circulation in the Very Low Birth Weight Infant | 240 | ||
| Physiologic Determinants of the Blood Pressure in the Very Low Birth Weight Infant (see also Chapter 1) | 241 | ||
| Clinical Determinants of Blood Pressure in the Very Low Birth Weight Infant | 242 | ||
| Gestational Age and Postnatal Age | 242 | ||
| Use of Antenatal Glucocorticoid Therapy | 243 | ||
| Blood Loss | 243 | ||
| Positive Pressure Ventilation | 243 | ||
| Patent Ductus Arteriosus (see also Chapter 13) | 244 | ||
| Systemic Vascular Resistance | 244 | ||
| Assessment of Cardiovascular Compromise in the Shocked Very Low Birth Weight Infant | 244 | ||
| Capillary Refill Time | 244 | ||
| Urine Output | 245 | ||
| Pulse Rate | 245 | ||
| Metabolic Acidosis/Lactic Acidosis | 245 | ||
| Blood Pressure (see also Chapter 3) | 245 | ||
| Cardiac Output (see also Chapters 1, 5, 6, and 10) | 246 | ||
| Monitoring of Peripheral and Mucosal Blood Flow | 246 | ||
| Pulse Oximeter Derived Perfusion Index | 246 | ||
| Systemic Blood Flow | 246 | ||
| Short- and Long-Term Effects of Cardiovascular Compromise/Shock in the Very Low Birth Weight Infant | 247 | ||
| Peri/Intraventricular Hemorrhage | 248 | ||
| Periventricular Leukomalacia | 248 | ||
| Long-Term Neurodevelopmental Outcome | 248 | ||
| Treatment Options in the Management of Cardiovascular Compromise/Shock in the Very Low Birth Weight Infant | 249 | ||
| Closing the Ductus Arteriosus | 249 | ||
| Volume Expansion | 249 | ||
| Vasopressor-Inotropes, Inotropes, and Lusitropes | 252 | ||
| Dopamine | 253 | ||
| Dobutamine | 255 | ||
| Epinephrine | 256 | ||
| Milrinone | 257 | ||
| Vasopressin | 257 | ||
| Treatment of Very Low Birth Weight Neonates with Vasopressor-Resistant Shock | 257 | ||
| Presentation and Management of Cardiovascular Compromise in the Very Low Birth Weight Infant on the First Postnatal Day | 258 | ||
| Transient Myocardial Dysfunction | 258 | ||
| Vasodilation and Hyperdynamic Myocardial Function | 260 | ||
| Perinatal Depression with Secondary Myocardial Dysfunction and/or Abnormal Peripheral Vasoregulation | 260 | ||
| Conclusion | 260 | ||
| References | 262 | ||
| Chapter 13 The Very Low Birth Weight Neonate with Hemodynamically Significant Ductus Arteriosus During the First Postnatal Week | 269 | ||
| Signs and Symptoms of Patent Ductus Arteriosus | 271 | ||
| Cardiovascular Adaptation to Patent Ductus Arteriosus | 272 | ||
| Effects of Hemodynamically Significant Patent Ductus Arteriosus on Blood Pressure | 273 | ||
| Effects of Hemodynamically Significant Patent Ductus Arteriosus on Organ Perfusion | 274 | ||
| Cerebral Blood Flow | 276 | ||
| Superior Mesenteric and Celiac Artery Blood Flow | 276 | ||
| Pulmonary Blood Flow | 277 | ||
| Changes in Cardiac Function Following PDA Ligation | 279 | ||
| Treatment | 280 | ||
| Surgical Ligation | 280 | ||
| Indomethacin | 281 | ||
| Ibuprofen | 282 | ||
| Indomethacin and Intracranial Hemorrhage | 282 | ||
| PDA and Neonatal Morbidity: To Treat or Not to Treat | 282 | ||
| Conclusion | 284 | ||
| References | 284 | ||
| Chapter 14 The Preterm Neonate with Cardiovascular and Adrenal Insufficiency | 293 | ||
| Adrenal Insufficiency | 293 | ||
| Cardiovascular Insufficiency: Hypotension and Shock (see also Chapter 1) | 297 | ||
| Evidence of AI in Ill Preterm Infants | 298 | ||
| Evidence of AI in Ill Late Preterm and Term Infants | 299 | ||
| Cardiovascular Insufficiency and Adrenal Insufficiency in Ill Infants | 300 | ||
| Mechanisms of Corticosteroids in the Treatment of Cardiovascular Insufficiency | 302 | ||
| Corticosteroid Therapy for Cardiovascular Insufficiency | 302 | ||
| Conclusion | 304 | ||
| References | 305 | ||
| Chapter 15 Shock in the Surgical Neonate | 311 | ||
| Definition and Phases of Neonatal Shock | 311 | ||
| Pathogenesis of Neonatal Shock | 312 | ||
| Diagnosis of Circulatory Compromise and Shock | 312 | ||
| Neonates with Surgical Condition and Shock | 312 | ||
| Respiratory Disorders | 312 | ||
| Congenital Diaphragmatic Hernia | 312 | ||
| Cystic Congenital Adenomatoid Malformation | 314 | ||
| Vascular Tumors | 315 | ||
| Hepatic Vascular Tumors | 315 | ||
| Sacrococcygeal Teratoma | 316 | ||
| Gastrointestinal Disorders | 316 | ||
| Gastroschisis and Omphalocele | 317 | ||
| Necrotizing Enterocolitis | 317 | ||
| References | 318 | ||
| Chapter 16 Hemodynamics and Brain Injury in the Preterm Neonate | 321 | ||
| Magnitude of Problem | 321 | ||
| Systemic and Cerebral Hemodynamic Vulnerability in Premature Infants | 322 | ||
| The Premature Cardiovascular System | 323 | ||
| Cerebral Hemodynamic Control in Premature Infants | 324 | ||
| Evidence for an Association Between Systemic Hemodynamic Disturbances and Prematurity-Related Brain Injury: Current Status | 325 | ||
| Blood Pressure and Prematurity-Related Brain Injury | 325 | ||
| Resolving the Relationship Between Systemic Hemodynamics and Prematurity-Related Brain Injury: Obstacles to Progress | 326 | ||
| Measurement of Relevant Hemodynamic and Metabolic Indices | 326 | ||
| Characterizing “Significant” Systemic Hemodynamic Insults Is Difficult In Sick Premature Infants | 327 | ||
| Establishing a Temporal Relationship Between Systemic Hemodynamic Changes and Brain Insults | 328 | ||
| Conclusion | 330 | ||
| References | 330 | ||
| Part II Fetal and Neonatal Cardiology | 341 | ||
| Section D Embryonic and Fetal Development | 341 | ||
| Chapter 17 The Genetics of Fetal and Neonatal Cardiovascular Disease | 343 | ||
| Genetic Basis of Congenital Heart Disease Wendy Chung, MD, PhD | 343 | ||
| Developments in Molecular Diagnostics for Congenital Heart Disease | 346 | ||
| What Is Involved with Genetic Testing? | 348 | ||
| What Is the Approach Used in Genetic Evaluation? | 349 | ||
| How to Interpret Test Results | 349 | ||
| References | 350 | ||
| The Developmental Biology and Genetics Underlying Human Heterotaxy | 350 | ||
| Left-Right Asymmetric Heart Anatomy | 351 | ||
| Theoretical Considerations in the Development of Chiral Asymmetry | 352 | ||
| The LRO Is a Conserved Ciliated Signaling Center | 353 | ||
| Structure and Function of Cilia | 354 | ||
| Asymmetric Gene Expression Downstream from the Left-Right Organizer | 356 | ||
| Clinical Implications: Genetics of Heterotaxy | 357 | ||
| Primary Ciliary Dyskinesia | 358 | ||
| Bardet-Biedl Syndrome | 358 | ||
| Nonsyndromic Htx | 358 | ||
| References | 360 | ||
| Genetics of Congenital Heart Disease | 362 | ||
| Nonsyndromic Congenital Heart Disease | 364 | ||
| Atrial Septal Defect | 364 | ||
| Ebstein Anomaly | 364 | ||
| Patent Ductus Arteriosus | 365 | ||
| Coarctation of the Aorta | 365 | ||
| Valvular Pulmonary Stenosis | 366 | ||
| Total Anomalous Pulmonary Venous Connections | 366 | ||
| Tetralogy of Fallot | 366 | ||
| Transposition of Great Arteries | 367 | ||
| Syndromic Congenital Heart Disease | 367 | ||
| Holt-Oram Syndrome | 367 | ||
| Noonan Syndrome | 368 | ||
| Alagille Syndrome | 368 | ||
| VATER Association | 369 | ||
| CHARGE Association | 370 | ||
| Kabuki Syndrome | 370 | ||
| Down Syndrome | 370 | ||
| Turner Syndrome | 371 | ||
| DiGeorge Syndrome | 372 | ||
| Williams Syndrome | 373 | ||
| Heterotaxy | 374 | ||
| References | 374 | ||
| Chapter 18 Human Cardiac Development in the First Trimester | 377 | ||
| Background | 378 | ||
| Specimens | 379 | ||
| Magnetic Resonance Imaging, Episcopic Fluorescence Image Capture, Processing, and Analysis | 379 | ||
| Cardiac Looping | 380 | ||
| Atrial Septation (Estimated Gestational Age -8 Weeks) | 383 | ||
| Ventricular Septation (Estimated Gestational Age Weeks) | 383 | ||
| Formation of the Atrioventricular Valves (Estimated Gestational Age -8weeks) | 384 | ||
| Outflow Septation and Semilunar Valve Morphogenesis (Estimated Gestational Age -8 Weeks) | 385 | ||
| Conclusion | 387 | ||
| Acknowledgments | 387 | ||
| References | 388 | ||
| Chapter 19 The Reappraisal of Normal and Abnormal Cardiac Development | 391 | ||
| How Does the Heart Develop? | 392 | ||
| Cardiac Looping | 395 | ||
| Formation of the Cardiac Chambers | 395 | ||
| Cardiac Septation | 399 | ||
| Development and Separation of the Outflow Tract | 403 | ||
| Relationship to Congenital Cardiac Malformations | 411 | ||
| Acknowledgment | 412 | ||
| References | 412 | ||
| Section E Fetal and Neonatal Cardiology | 415 | ||
| Chapter 20 New Concepts for Training the Pediatric Cardiology Workforce of the Future | 417 | ||
| Background and Significance | 417 | ||
| Challenges Facing the Health Workforce Today | 418 | ||
| Inadequacy of the Current Health Care Delivery System | 419 | ||
| The Health Care System of the Future | 420 | ||
| Patients | 420 | ||
| Community Health Workers and Grand-Aides | 421 | ||
| Nurses | 421 | ||
| Physicians | 422 | ||
| Training the New Health Professionals: Questioning Assumptions | 422 | ||
| How Much Do Patients Want to Know? | 422 | ||
| Are We Training for Teamwork? | 423 | ||
| Do Physicians Need to Be Well Rounded? How Much Education Is Necessary? | 423 | ||
| Interprofessional Education? | 425 | ||
| The Ideal Team | 425 | ||
| A Futuristic Ideal: The College for Health | 426 | ||
| Final Thoughts | 427 | ||
| References | 427 | ||
| Chapter 21 The Current Role of Fetal Echocardiography | 431 | ||
| Conclusion | 441 | ||
| Reference | 441 | ||
| Chapter 22 Clinical Evaluation of Cardiovascular Function in the Human Fetus | 443 | ||
| The Fetal Circulation | 443 | ||
| Factors Affecting Fetal Cardiac Output | 443 | ||
| The Transitional Circulation | 444 | ||
| The Etiology of Hydrops Fetalis | 444 | ||
| Mechanisms of Fetal Congestive Heart Failure | 444 | ||
| Increased Afterload | 444 | ||
| Twin-Twin Transfusion | 444 | ||
| Increased Preload | 445 | ||
| Arteriovenous Fistula | 445 | ||
| External Compression | 446 | ||
| Prognosis of Fetal Heart Failure—Markers of Fetal Mortality | 446 | ||
| Introduction to the Use of the Cardiovascular Profile Score (Table 22-2) | 446 | ||
| Ventricular Function in the Fetus | 446 | ||
| Fetal Congestive Heart Failure | 446 | ||
| Cardiovascular Profile Score in Ebstein’s Anomaly | 448 | ||
| Treatment of Fetal Heart Failure | 448 | ||
| Digoxin | 449 | ||
| Digoxin Treatment | 450 | ||
| Outcome | 450 | ||
| Effect of Digoxin on CVPS | 450 | ||
| Differences in CVPS Between Survivors and Nonsurvivors | 450 | ||
| References | 451 | ||
| Chapter 23 Cardiac Surgery in the Neonate with Congenital Heart Disease | 453 | ||
| Palliative Operations | 454 | ||
| Pulmonary Artery Banding | 454 | ||
| Aortopulmonary Shunts (Blalock-Taussig Shunt) | 455 | ||
| Specific Lesions | 456 | ||
| Left-to-Right Shunt Lesions | 456 | ||
| Ventricular Septal Defect | 456 | ||
| Patent Ductus Arteriosus of Botalli | 456 | ||
| Truncus Arteriosus | 458 | ||
| Aortopulmonary Window | 458 | ||
| Obstructive Lesions | 458 | ||
| Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum | 458 | ||
| Aortic Stenosis | 459 | ||
| Coarctation of the Aorta | 460 | ||
| Interrupted Aortic Arch | 462 | ||
| Hypoplastic Left Heart Syndrome | 463 | ||
| Cyanotic Heart Lesions | 463 | ||
| Transposition of the Great Arteries | 463 | ||
| Total Anomalous Pulmonary Venous Connection | 465 | ||
| Mechanical Circulatory Support in the Neonate | 467 | ||
| Conclusion | 470 | ||
| References | 470 | ||
| Chapter 24 Regional Blood Flow Monitoring in the Perioperative Period | 473 | ||
| Historical Perspective and Technologic Development | 473 | ||
| Descriptive Physiology of Multiple Site Near-Infrared Spectroscopy | 474 | ||
| Goal-Directed Global Hemodynamic Management with Multisite Near-Infrared Spectroscopy | 476 | ||
| Perioperative Cerebral Oxygenation and Function | 480 | ||
| Perioperative Somatic Oxygenation and Organ Function | 481 | ||
| Interstage and Home Monitoring | 484 | ||
| Conclusion | 484 | ||
| References | 485 | ||
| Chapter 25 Mechanical Pump Support and Cardiac Transplant in the Neonate | 489 | ||
| Neonatal Heart Transplantation | 489 | ||
| History | 489 | ||
| Indication for Transplant | 490 | ||
| Unique Aspects of Heart Transplantation in Infants | 490 | ||
| Waitlist Mortality | 490 | ||
| ABO Incompatible Transplants | 491 | ||
| Surgical Approach and Mortality | 492 | ||
| Immunosuppression and Rejection | 492 | ||
| Mechanical Circulatory Support in the Infant | 493 | ||
| Indications for Support | 493 | ||
| Extracorporeal Membrane Oxygenation | 494 | ||
| Ventricular Assist Device | 495 | ||
| Pulsatile Flow Devices | 495 | ||
| Continuous Flow Devices | 497 | ||
| Future | 498 | ||
| PumpKIN | 499 | ||
| References | 500 | ||
| Chapter 26 Catheter-Based Therapy in the Neonate with Congenital Heart Disease | 503 | ||
| Vascular Access | 503 | ||
| Balloon Atrial Septostomy | 504 | ||
| Atrial Septoplasty | 506 | ||
| Septoplasty with Interatrial Stent Placement | 508 | ||
| Pulmonary Balloon Valvuloplasty in the Neonate | 508 | ||
| Perforation of Membranous Pulmonary Valve in Pulmonary Atresia and Intact Ventricular Septum | 510 | ||
| Ductal Stenting | 513 | ||
| Balloon Aortic Valvuloplasty in the Neonate | 515 | ||
| Transcatheter Management for Neonatal Coarctation | 517 | ||
| Other Catheter-Based Therapies for Neonates with Congenital Heart Disease | 517 | ||
| Techniques on the Horizon | 518 | ||
| Conclusion | 518 | ||
| Acknowledgment | 518 | ||
| References | 518 | ||
| Chapter 27 Hybrid Management Techniques in the Treatment of the Neonate with Congenital Heart Disease | 521 | ||
| Hypoplastic Left Heart Syndrome | 521 | ||
| Historical Perspective | 521 | ||
| Triumphs and Pitfalls | 524 | ||
| Future Potential | 525 | ||
| Perventricluar Ventricular Septal Defect Closure | 527 | ||
| Adjustable Pulmonary Artery Bands | 528 | ||
| Intraoperative Angiocardiography | 530 | ||
| Conclusion | 533 | ||
| References | 533 | ||
| Index | 537 | ||
| A | 537 | ||
| B | 538 | ||
| C | 539 | ||
| D | 541 | ||
| E | 542 | ||
| F | 542 | ||
| G | 543 | ||
| H | 543 | ||
| I | 544 | ||
| J | 545 | ||
| K | 545 | ||
| L | 545 | ||
| M | 545 | ||
| N | 546 | ||
| O | 547 | ||
| P | 547 | ||
| Q | 549 | ||
| R | 549 | ||
| S | 549 | ||
| T | 550 | ||
| U | 550 | ||
| V | 550 | ||
| W | 551 | ||
| X | 551 | ||
| Y | 551 | ||
| Z | 551 |