Chapter
Part I Neonatal Hemodynamics
Section A Principles of Developmental Cardiovascular Physiology and Pathophysiology
Chapter 1 Principles of Developmental Cardiovascular Physiology and Pathophysiology
Principles of Developmental Physiology
Pressure, Flow, and Resistance
Organ Blood Flow Distribution
Microcirculatory Physiology
Myocardial Function—Developmental Aspects
Impact of the Immature Autonomic Nervous System on Regulating Cardiac Function and Vascular Tone
Developmental Cardiovascular Pathophysiology: Etiology and Pathophysiology of Neonatal Shock
Definition and Phases of Shock
Etiology of Neonatal Shock
Adrenal Insufficiency (See Chapters 12 and 14)
Downregulation of Adrenergic Receptors
Chapter 2 Autoregulation of Vital and Nonvital Organ Blood Flow in the Preterm and Term Neonate
Regulation of Arterial Tone
The Role of Conduit Arteries in Regulating Vascular Resistance
Arterial Reaction to Pressure (Autoregulation)
Interaction of Autoregulation and Hypoxic Vasodilatation
Interaction of Autoregulation and Pco2
Interaction of Autoregulation and Functional Activation (Metabolic Blood Flow Control)
Flow-mediated Vasodilatation
Sympathetic Nervous System
Humoral Factors in General Circulation
Autoregulation of Cerebral Blood Flow in the Immature Brain
Effect of Carbon Dioxide on Cerebral Blood Flow
Metabolic Control of Blood Flow to the Brain
Adrenergic Mechanisms Affecting Cerebral Blood Flow
Effect of Medications on Cerebral Blood Flow
Ischemic Thresholds in the Brain
Blood Flow to Other Organs
Distribution of Cardiac Output in the Healthy Human Neonate
Blood Flow to the Upper Part of the Body
Blood Flow to the Lower Part of the Body
Mechanisms Governing the Redistribution of Cardiac Output in the Fetal ‘“Dive” Reflex
Distribution of Cardiac Output in the Shocked Newborn
The Term Neonate with Low Cardiac Output
The Very Preterm Neonate During Immediate Postnatal Adaptation
Chapter 3 Definition of Normal Blood Pressure Range:
Direct Measurement of Blood Pressure
Noninvasive Measurement of Blood Pressure
Normative Data for Blood Pressure in Neonates
Adjuncts to Blood Pressure Measurement in the Diagnosis of Compromised Circulatory Function
Capillary Refill Time and Central-Peripheral Temperature Difference
Clinical Factors that May Affect Blood Pressure
Maternal Age and Blood Pressure
Time of Umbilical Cord Clamping
Other Indicators of Changes in Circulatory Function
Section B Diagnosis of Neonatal Shock: Methods and Their Clinical Applications
Chapter 4 Methods to Assess Systemic and Organ Blood Flow in the Neonate
Near-Infrared Spectroscopy
Algorithms and Wavelength
Quantification of Cerebral Blood Flow
Reproducibility and Validation
Indocyanine Green as an Alternative Tracer
Trend Monitoring of Hemoglobin Signals
The Hemoglobin Difference, or Oxygenation Index
Coherence of the Spontaneous Variability of Cerebral Hemoglobin Oxygenation and Variability of Arterial Blood Pressure as an Indication of Cerebral Autoregulation
Diffuse Correlation Spectroscopy
Quantification of Hemoglobin-Oxygen Saturation
Bias of Tissue Oxygen Saturation
Precision of the Tissue Oxygen Saturation
Importance of the Low Precision of the Tissue Oxygen Saturation
Magnetic Resonance Imaging
Measurement of Blood Flow
Qualitative Flow Imaging (Fig. 4-3)
Quantitative Flow Imaging
Single Photon Emission Computed Tomography
Stable Xenon-Enhanced Computed Tomography
Positron Emission Tomography
Measurement of Flow to Other Organs
Chapter 5 Functional Echocardiography in the Neonatal Intensive Care Unit
Two-Dimensional Imaging and Normal Cardiac Structure
What Can Be Measured with Functional Echocardiography in the Neonatal Intensive Care Units?
Ductal Patency and Direction of Shunting
Determination of Hemodynamic Significance
Natural History of Postnatal Constriction
Pulmonary Artery Pressure
Pulmonary Artery Pressure from a Ductal Shunt
Pulmonary Artery Pressure from Tricuspid Incompetence
Pulmonary Artery Doppler Time to Peak Velocity
Measurement of Blood Flow and Cardiac Output
Left Pulmonary Artery Velocities: Pulmonary Blood Flow
Myocardial Function Measures
Myocardial Function: Future Developments
Functional Echocardiography in the Neonatal Intensive Care Units in Specific Clinical Situations
Very Preterm Baby During the Transitional Period
Measurement of Systemic Blood Flow
Assessment of Early Ductal Constriction and Shunt Direction
Preterm Infant with Suspected PDA
Baby with Clinically Suspected Circulatory Compromise
Baby with Suspected Primary Persistent Pulmonary Hypertension of the Newborn
Chapter 6 Assessment of Cardiac Output in Neonates:
Oxygen Fick (O2-Fick) Method
Pulmonary Oxygen Uptake (VO2)
Oxygen Concentration Gradient (CaO2 − CvO2)
Cardiac Index (Calculation Examples)
Carbon Dioxide Fick (CO2-Fick) Method
Modified Carbon Dioxide Fick Method (mCO2F)
Carbon Dioxide Rebreathing Technology (CO2R)
Obtaining the Impedance Cardiogram
Measurement of Thoracic Electrical Bio-impedance
Bio-impedance and Bio-reactance
Interpretation of the Impedance Cardiogram
Impedance Cardiography/Impedance Plethysmography
Alignment of Erythrocytes and Electrical Velocimetry
Chapter 7 Near-Infrared Spectroscopy and Its Use for the Assessment of Tissue Perfusion in the Neonate
Principles of Near-Infrared Spectroscopy
Near-Infrared Spectrophotometers
Continuous Wave Instruments
Spatially Resolved Spectroscopy
Time-of-flight Instruments
Frequency Domain Instruments
Measurements of Physiologic Variables
Cerebral Venous Oxygen Saturation
Peripheral Venous Oxygen Saturation
Physiologic Observations Using Near-Infrared Spectroscopy
Factors Determining Oxygen Delivery
Cerebral Blood Flow (CBF)
Cerebral Venous Oxygen Saturation and Consumption
Peripheral Venous Oxygen Saturation and Consumption
Fractional Oxygen Extraction
Oxygen Delivery–Consumption Coupling
Early Postnatal Adaptation
Chapter 8 Clinical Applications of Near-Infrared Spectroscopy in Neonates
Feasibility of Near-Infrared Spectroscopy-Monitored rScO2 and cFTOE in Clinical Practice in the NICU
Application of the Sensor and Its Pitfalls
Relation to Other Monitoring Devices
Clinical Conditions Associated with Low rScO2
Clinical Conditions Associated with High rScO2 Values
Chapter 9 Advanced Magnetic Resonance Neuroimaging Techniques in the Neonate with a Focus on Hemodynamic-Related Brain Injury
Magnetic Resonance–Compatible Neonatal Incubator
Magnetic Resonance–Compatible Video Monitoring System
Diffusion-Weighted Imaging and Diffusion Tensor Imaging of the Neonatal Brain
Perfusion Imaging of the Neonatal Brain
Exogenous Contrast Agents
Blood Oxygen Level–Dependent Functional Magnetic Resonance Imaging
Continuous Arterial Spin Labeling
Pulsed Arterial Spin Labeling
Quantitative Proton Magnetic Resonance Spectroscopy of the Neonatal Brain
Chapter 10 Cardiovascular Magnetic Resonance in the Study of Neonatal Hemodynamics
Current Understanding of Neonatal Hemodynamics
Current Cotside Circulatory Assessment
Optimal Circulatory Management
Current Cardiovascular Magnetic Resonance Imaging
Success of Cardiovascular Magnetic Resonance in the Adult Population
Performing Cardiovascular Magnetic Resonance Imaging in the Newborn
Adapting Cardiovascular Magnetic Resonance for Use in Newborns
Cine Cardiovascular Magnetic Resonance
Phase Contrast Cardiovascular Magnetic Resonance
Emerging Cardiac Magnetic Resonance Imaging
3-D Visualization of Flow with Phase Contrast
Assessment of Myocardial Motion
Potential Role of Cardiovascular Magnetic Resonance in the Study of Neonatal Hemodynamics
Definition of the Pathophysiology of Circulatory Failure
Acting as a Biomarker in Studies Assessing the Cardiovascular Effects of Vasopressor/Inotropes, Inotropes, and Lusitropes
Guiding the Development of Emerging Echocardiographic Techniques
Advantages and Disadvantages of Functional Cardiovascular Magnetic Resonance Imaging
Chapter 11 Assessment of the Microcirculation in the Neonate
Why Assess the Microcirculation?
Where to Study the Microcirculation in the Human Newborn?
Assessment of Skin Microcirculation Responses
Postocclusive Reactive Hyperemia
Microvasculature of the Preterm Neonate Studied by LDF
Peripheral Microvascular Blood Flow and Neonatal Gender
Mechanisms of Preterm Microvascular Control
Vasodilators Regulating Microvascular Tone
Vasoconstrictors Regulating Microvascular Tone
Retinography and Cardiovascular Programming
Future Applications in Neonatal Medicine
Section C Clinical Presentations and Relevance of Neonatal Shock
Chapter 12 Clinical Presentations of Neonatal Shock:
Definition of Hypotension and its Relationship to Low Systemic Perfusion
The Transitional Circulation in the Very Low Birth Weight Infant
Physiologic Determinants of the Blood Pressure in the Very Low Birth Weight Infant (see also Chapter 1)
Clinical Determinants of Blood Pressure in the Very Low Birth Weight Infant
Gestational Age and Postnatal Age
Use of Antenatal Glucocorticoid Therapy
Positive Pressure Ventilation
Patent Ductus Arteriosus (see also Chapter 13)
Systemic Vascular Resistance
Assessment of Cardiovascular Compromise in the Shocked Very Low Birth Weight Infant
Metabolic Acidosis/Lactic Acidosis
Blood Pressure (see also Chapter 3)
Cardiac Output (see also Chapters 1, 5, 6, and 10)
Monitoring of Peripheral and Mucosal Blood Flow
Pulse Oximeter Derived Perfusion Index
Short- and Long-Term Effects of Cardiovascular Compromise/Shock in the Very Low Birth Weight Infant
Peri/Intraventricular Hemorrhage
Periventricular Leukomalacia
Long-Term Neurodevelopmental Outcome
Treatment Options in the Management of Cardiovascular Compromise/Shock in the Very Low Birth Weight Infant
Closing the Ductus Arteriosus
Vasopressor-Inotropes, Inotropes, and Lusitropes
Treatment of Very Low Birth Weight Neonates with Vasopressor-Resistant Shock
Presentation and Management of Cardiovascular Compromise in the Very Low Birth Weight Infant on the First Postnatal Day
Transient Myocardial Dysfunction
Vasodilation and Hyperdynamic Myocardial Function
Perinatal Depression with Secondary Myocardial Dysfunction and/or Abnormal Peripheral Vasoregulation
Chapter 13 The Very Low Birth Weight Neonate with Hemodynamically Significant Ductus Arteriosus During the First Postnatal Week
Signs and Symptoms of Patent Ductus Arteriosus
Cardiovascular Adaptation to Patent Ductus Arteriosus
Effects of Hemodynamically Significant Patent Ductus Arteriosus on Blood Pressure
Effects of Hemodynamically Significant Patent Ductus Arteriosus on Organ Perfusion
Superior Mesenteric and Celiac Artery Blood Flow
Changes in Cardiac Function Following PDA Ligation
Indomethacin and Intracranial Hemorrhage
PDA and Neonatal Morbidity: To Treat or Not to Treat
Chapter 14 The Preterm Neonate with Cardiovascular and Adrenal Insufficiency
Cardiovascular Insufficiency: Hypotension and Shock (see also Chapter 1)
Evidence of AI in Ill Preterm Infants
Evidence of AI in Ill Late Preterm and Term Infants
Cardiovascular Insufficiency and Adrenal Insufficiency in Ill Infants
Mechanisms of Corticosteroids in the Treatment of Cardiovascular Insufficiency
Corticosteroid Therapy for Cardiovascular Insufficiency
Chapter 15 Shock in the Surgical Neonate
Definition and Phases of Neonatal Shock
Pathogenesis of Neonatal Shock
Diagnosis of Circulatory Compromise and Shock
Neonates with Surgical Condition and Shock
Congenital Diaphragmatic Hernia
Cystic Congenital Adenomatoid Malformation
Gastrointestinal Disorders
Gastroschisis and Omphalocele
Necrotizing Enterocolitis
Chapter 16 Hemodynamics and Brain Injury in the Preterm Neonate
Systemic and Cerebral Hemodynamic Vulnerability in Premature Infants
The Premature Cardiovascular System
Cerebral Hemodynamic Control in Premature Infants
Evidence for an Association Between Systemic Hemodynamic Disturbances and Prematurity-Related Brain Injury: Current Status
Blood Pressure and Prematurity-Related Brain Injury
Resolving the Relationship Between Systemic Hemodynamics and Prematurity-Related Brain Injury: Obstacles to Progress
Measurement of Relevant Hemodynamic and Metabolic Indices
Characterizing “Significant” Systemic Hemodynamic Insults Is Difficult In Sick Premature Infants
Establishing a Temporal Relationship Between Systemic Hemodynamic Changes and Brain Insults
Part II Fetal and Neonatal Cardiology
Section D Embryonic and Fetal Development
Chapter 17 The Genetics of Fetal and Neonatal Cardiovascular Disease
Genetic Basis of Congenital Heart Disease Wendy Chung, MD, PhD
Developments in Molecular Diagnostics for Congenital Heart Disease
What Is Involved with Genetic Testing?
What Is the Approach Used in Genetic Evaluation?
How to Interpret Test Results
The Developmental Biology and Genetics Underlying Human Heterotaxy
Left-Right Asymmetric Heart Anatomy
Theoretical Considerations in the Development of Chiral Asymmetry
The LRO Is a Conserved Ciliated Signaling Center
Structure and Function of Cilia
Asymmetric Gene Expression Downstream from the Left-Right Organizer
Clinical Implications: Genetics of Heterotaxy
Primary Ciliary Dyskinesia
Genetics of Congenital Heart Disease
Nonsyndromic Congenital Heart Disease
Valvular Pulmonary Stenosis
Total Anomalous Pulmonary Venous Connections
Transposition of Great Arteries
Syndromic Congenital Heart Disease
Chapter 18 Human Cardiac Development in the First Trimester
Magnetic Resonance Imaging, Episcopic Fluorescence Image Capture, Processing, and Analysis
Atrial Septation (Estimated Gestational Age -8 Weeks)
Ventricular Septation (Estimated Gestational Age Weeks)
Formation of the Atrioventricular Valves (Estimated Gestational Age -8weeks)
Outflow Septation and Semilunar Valve Morphogenesis (Estimated Gestational Age -8 Weeks)
Chapter 19 The Reappraisal of Normal and Abnormal Cardiac Development
How Does the Heart Develop?
Formation of the Cardiac Chambers
Development and Separation of the Outflow Tract
Relationship to Congenital Cardiac Malformations
Section E Fetal and Neonatal Cardiology
Chapter 20 New Concepts for Training the Pediatric Cardiology Workforce of the Future
Background and Significance
Challenges Facing the Health Workforce Today
Inadequacy of the Current Health Care Delivery System
The Health Care System of the Future
Community Health Workers and Grand-Aides
Training the New Health Professionals: Questioning Assumptions
How Much Do Patients Want to Know?
Are We Training for Teamwork?
Do Physicians Need to Be Well Rounded? How Much Education Is Necessary?
Interprofessional Education?
A Futuristic Ideal: The College for Health
Chapter 21 The Current Role of Fetal Echocardiography
Chapter 22 Clinical Evaluation of Cardiovascular Function in the Human Fetus
Factors Affecting Fetal Cardiac Output
The Transitional Circulation
The Etiology of Hydrops Fetalis
Mechanisms of Fetal Congestive Heart Failure
Prognosis of Fetal Heart Failure—Markers of Fetal Mortality
Introduction to the Use of the Cardiovascular Profile Score (Table 22-2)
Ventricular Function in the Fetus
Fetal Congestive Heart Failure
Cardiovascular Profile Score in Ebstein’s Anomaly
Treatment of Fetal Heart Failure
Effect of Digoxin on CVPS
Differences in CVPS Between Survivors and Nonsurvivors
Chapter 23 Cardiac Surgery in the Neonate with Congenital Heart Disease
Aortopulmonary Shunts (Blalock-Taussig Shunt)
Left-to-Right Shunt Lesions
Ventricular Septal Defect
Patent Ductus Arteriosus of Botalli
Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum
Hypoplastic Left Heart Syndrome
Transposition of the Great Arteries
Total Anomalous Pulmonary Venous Connection
Mechanical Circulatory Support in the Neonate
Chapter 24 Regional Blood Flow Monitoring in the Perioperative Period
Historical Perspective and Technologic Development
Descriptive Physiology of Multiple Site Near-Infrared Spectroscopy
Goal-Directed Global Hemodynamic Management with Multisite Near-Infrared Spectroscopy
Perioperative Cerebral Oxygenation and Function
Perioperative Somatic Oxygenation and Organ Function
Interstage and Home Monitoring
Chapter 25 Mechanical Pump Support and Cardiac Transplant in the Neonate
Neonatal Heart Transplantation
Indication for Transplant
Unique Aspects of Heart Transplantation in Infants
ABO Incompatible Transplants
Surgical Approach and Mortality
Immunosuppression and Rejection
Mechanical Circulatory Support in the Infant
Extracorporeal Membrane Oxygenation
Ventricular Assist Device
Chapter 26 Catheter-Based Therapy in the Neonate with Congenital Heart Disease
Balloon Atrial Septostomy
Septoplasty with Interatrial Stent Placement
Pulmonary Balloon Valvuloplasty in the Neonate
Perforation of Membranous Pulmonary Valve in Pulmonary Atresia and Intact Ventricular Septum
Balloon Aortic Valvuloplasty in the Neonate
Transcatheter Management for Neonatal Coarctation
Other Catheter-Based Therapies for Neonates with Congenital Heart Disease
Techniques on the Horizon
Chapter 27 Hybrid Management Techniques in the Treatment of the Neonate with Congenital Heart Disease
Hypoplastic Left Heart Syndrome
Perventricluar Ventricular Septal Defect Closure
Adjustable Pulmonary Artery Bands
Intraoperative Angiocardiography