Visual Guide to Neonatal Cardiology E-Book

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 1101

Veröffentlichungsjahr: 2018

Beschreibung

The Visual Guide to Neonatal Cardiology is a comprehensive, highly illustrated, reference covering the evaluation, diagnosis and management of cardiac disease in the newborn.

• Contains over 900 color illustrations, including patient photographs, chest roentgenograms, electrocardiograms, echocardiograms, angiocardiograms, 3D computed tomogramphy, magnetic resonance imaging, pathologic specimens, and other relevant visual aids
• Discusses the natural history of fetal heart disease and the rationale, indications, technique, and impact of fetal cardiac intervention
• Reviews the anatomy and physiology of the neonatal cardiovascular system, including differences within the fetal, transitional, neonatal, child and adult circulatory system
• Highlights key steps for taking a patient history, including detailed discussion of the cardiac examination (inspection, palpation and auscultation of heart sounds and murmurs)
• Presents over 35 morphologic conditions with sections covering introduction, epidemiology, etiology with accepted or postulated embryogenesis, pathophysiology, clinical presentation, physical examination findings, diagnostic evaluation, management, and prognosis
• Includes a neonatal formulary reviewing selected medications currently used for treatment of perioperative low cardiac output, congestive heart failure, pulmonary hypertension, sedation, pain and anticoagulation in neonates

Leseprobe

Table of Contents

Cover

Title Page

Copyright

Dedication

Preface

List of Contributors

Part I: Prenatal and Perinatal Issues

Chapter 1: Cardiac Embryology and Embryopathy

Initial Stages of Development

Looping of the Heart Tube

The Process of Ballooning

Formation of the Atrial Chambers

Atrial Septation

Ventricular Development

Development and Maldevelopment of the Outflow Tract

Development of the Coronary Circulation

Acknowledgments

References

Chapter 2: Maternal, Familial, and Non-Cardiac Fetal Conditions Affecting the Fetal and Neonatal Heart

Maternal Factors

Twins

Familial

Non-cardiac Fetal Conditions

References

Chapter 3: The Natural and Unnatural History of Fetal Heart Disease

References

Part II: General Neonatal Issues

Chapter 4: Epidemiology of Heart Defects

Prevalence of Individual Lesions

Changes in Prevalence Over Time

Regional and Racial Variation

Impact of Fetal Testing

Non-Genetic Risk Factors

References

Chapter 5: The Transitional and Neonatal Heart and Cardiovascular System

Birth and Neonatal Period

Acknowledgment

References

Chapter 6: History and Physical Examination

History

Physical Examination

Further Reading

Chapter 7: The Cyanotic Newborn

Reference

Further Reading

Chapter 8: The Tachypneic Newborn

Reference

Further Reading

Chapter 9: The Hypoperfused Newborn

Etiology

History

Physical Examination

Diagnostic Tests

Management

Further Reading

Chapter 10: The Dysmorphic Newborn

Further Reading

Part III: Diagnostic Procedures

Chapter 11: Chest Roentgenogram

Pulmonary Vascularity

Cardiomegaly

Situs

Great Vessels

Extracardiac Evaluation

Lines and Tubes in Patients With Congenital Heart Disease

Complications

Chapter 12: Electrocardiogram

ECG Leads

Basic ECG Setup

Wave Morphologies

ECG Intervals

Heart Rate

Cardiac Rhythm

Axis

Right-sided Chest Leads

Abnormalities on the ECG

Further Reading

Chapter 13: Echocardiogram

History

Physics

Performance of a Pediatric Echocardiogram

References

Chapter 14: Cardiac Catheterization and Angiocardiography

Patent Ductus Arteriosus (Figures 14.1 and 14.2)

Pulmonary Valve Stenosis (Figure 14.3)

Critical Aortic Valve Stenosis (Figure 14.4)

Coarctation of the Aorta (Figure 14.5)

Major Aorto-Pulmonary Collateral Arteries (Figure 14.6)

Transposition of the Great Arteries (Figure 14.7)

Hypoplastic Left Ventricle (Figure 14.8)

Total Anomalous Pulmonary Venous Connection (Figure 14.9)

Rotational Angiography (Figure 14.10)

References

Chapter 15: Computed Tomography

Scanning Technique for Cardiac CTA in Neonates

Advantages of Cardiac CTA Over Other Imaging Modalities

Postprocessing of Cardiac CTA

Further Reading

Chapter 16: Magnetic Resonance Imaging

Indications for Neonatal CMR

References

Chapter 17: Electrophysiologic Testing, Transesophageal Pacing and Pacemakers

Pacemakers

Implantable Cardioverter-Defibrillators

Electrophysiology Studies

Transesophageal and Temporary Pacing

Reference

Part IV: Specific Morphologic Conditions

Chapter 18: Total Anomalous Pulmonary Venous Connection

Definition

Etiology

Embryologic Basis

Anatomy

Pathophysiology

Total Anomalous Pulmonary Venous Drainage

Treatment

References

Chapter 19: Other Anomalies of Pulmonary and Systemic Venous Connections

Achnowledgment

References

Chapter 20: Anomalies of Atrial Septation

Background and Anatomy

Neonatal Presentation

Management and Treatment Options

References

Chapter 21: Atrial Chamber Obstruction

Cor Triatriatum Sinister

Cor Triatriatum Dexter

References

Chapter 22: Common Atrioventricular Canal Defects

Pathophysiology

Clinical Manifestation

Investigations

Management

References

Chapter 23: Ventricular Septal Defect

Pathophysiology

Clinical Presentation and Diagnosis

Nomenclature and Anatomy

Management

References

Chapter 24: Tricuspid Atresia

Anatomy

Physiology

Physical Examination

Electrocardiogram

Chest X-Ray

Echocardiography

Cardiac Catheterization

Preoperative Management

Surgical Management

Postoperative Management

References

Chapter 25: Ebstein Malformation and Tricuspid Valve Dysplasias

Ebstein Malformation

Tricuspid Valve Dysplasia

References

Chapter 26: Pulmonary Valve and Pulmonary Arterial Stenosis

Pulmonary Valve Stenosis

Pulmonary Arterial Stenosis

References

Chapter 27: Pulmonary Atresia with Intact Ventricular Septum

Embryology

Pathology

Physical Examination

Chest X-ray

Electrocardiography

Echocardiography

Cardiac Catheterization

Medical Therapy

Surgery

Transcatheter Therapy

Outcomes

Conclusions

References

Chapter 28: Tetralogy of Fallot with Pulmonary Stenosis or Atresia

Tetralogy of Fallot with Pulmonary Stenosis

Pulmonary Valve Atresia with Ventricular Septal Defect (Tetralogy of Fallot with Pulmonary Atresia)

Further Reading

Chapter 29: Absent Pulmonary Valve

References

Chapter 30: Transposition of the Great Arteries

References

Chapter 31: Congenitally Corrected Transposition of the Great Arteries

Morphology and Associated Lesions

Clinical Presentation

Outcomes and Interventions

References

Chapter 32: Common Arterial Trunk (Truncus Arteriosus)

References

Chapter 33: Mitral Valve Apparatus Abnormalities

Normal Mitral Valve Complex

Embryology

Abnormalities of Mitral Valve Apparatus

Conclusions

References

Chapter 34: Hypoplastic Left Heart Syndrome

Anatomy

Physiology

Diagnosis

Management

Prognosis

References

Chapter 35: Aortic Stenosis

Aortic Valve Stenosis

Subaortic Stenosis

References

Further Reading

Chapter 36: Coronary Artery Anomalies

References

Chapter 37: Aorto-Left Ventricular Tunnel

Aorto-Left Ventricular Tunnel: Anatomic Characteristics

Associated Anomalies

Clinical Presentation

Diagnostic Methods

Management

Conclusions

References

Chapter 38: Coronary Cameral Fistulas

Definition

Etiology

Pathophysiology and Natural History

Diagnosis

Management Strategies

Interventional Catheterization

Surgical Therapy

Conclusions

References

Chapter 39: Aortopulmonary Window

Reference

Further Reading

Chapter 40: Anomalous Origin of a Branch Pulmonary Artery From the Ascending Aorta (Hemitruncus)

Further Reading

Chapter 41: Interrupted Aortic Arch

References

Chapter 42: Coarctation of the Aorta

Pathophysiology

Clinical Manifestation

Investigations

Management

References

Chapter 43: Vascular Rings and Pulmonary Slings

Anomalies of the Aortic Arch

Vascular Rings

Anomalous Subclavian Artery (Kommerell diverticulum)

Diagnosis

Pulmonary Artery Slings

References

Chapter 44: Double Outlet Right Ventricle

Acknowledgments

References

Chapter 45: Double Outlet Left Ventricle

Etiology

Morphology

Pathophysiology

Surgical Repair

Long-term Outcomes

References

Chapter 46: Single Ventricle and Biventricular Hearts with Hypoplasia of One Ventricle

Embryology and Genetics

Prenatal Circulation

Postnatal Circulation and Clinical Presentation

Preoperative Evaluation

Management

Surgical Strategies

Outcomes

Conclusions

References

Chapter 47: Dextrocardia and the Heterotaxy Syndromes

Heterotaxy Syndrome

Definitions

Associated Non-Cardiac Anomalies

Associated Cardiac Anomalies

Management

Outcomes

Conclusions

References

Chapter 48: Ectopia Cordis and Thoracopagus Twins

Ectopia Cordis

Thoracopagus Twins

References

Chapter 49: Patent Ductus Arteriosus

Clinical Findings

Imaging

Management

References

Chapter 50: Neonatal Hypertrophic Cardiomyopathy and Syndromes with Infantile Cardiac Hypertrophy

Hypertrophic Cardiomyopathy

Common Causes of Cardiac Hypertrophy in Neonates

Characteristics of Neonatal HCM and Syndromes with Concomitant Cardiac Hypertrophy

Genetics of Neonatal HCM, Noonan Syndrome, and Pompe Disease

Survival and Outcomes in Neonatal HCM and Syndromes with Concomitant LVH

Conclusions

References

Chapter 51: Dilated Cardiomyopathy and Myocarditis

Etiology

Presentation

Testing

Neonatal Myocarditis

References

Chapter 52: Cardiac Chamber Aneurysms and Diverticula

Ventricular Diverticula and Aneurysms

Atrial Diverticula and Aneurysms

References

Chapter 53: Cardiac Tumors

Cardiac Rhabdomyoma

Intrapericardial Teratoma

Fibromas

Cardiac Hemangioma

References

Chapter 54: Arteriovenous Malformations

Intracranial

Extracranial Arteriovenous Malformations

Conclusions

Acknowledgement

References

Chapter 55: Pericardial Defects

Manifestations

Diagnosis

Treatment

Ectopia Cordis

Treatment

References

Chapter 56: Miscellaneous Chest Abnormalities Affecting the Heart: Diaphragmatic Hernia and Eventration; Congenital Cystic Adenomatoid Malformation of the Lung

Diaphragmatic Hernia

Eventration and Diaphragm Paresis

Congenital Cystic Adenomatoid Malformation of the Lung (Congenital Pulmonary Airway Malformation)

References

Chapter 57: Persistent Pulmonary Hypertension of the Newborn

Circulatory Pathophysiology

Diagnostic Work-up

Management Considerations

Conclusions

References

Chapter 58: Hydrops Fetalis

Presentation

Causes

Evaluation

Treatment

Maternal Complications

Prognosis

Fetal Hydrops for the Perinatal Cardiologist

Counseling

Antepartum

Delivery

CV Profile Score in Hydrops

Cardiomegaly

Abnormal Myocardial Function

Arterial Doppler Redistribution of Fetal Cardiac Output

Cardiovascular Profile Score

Conclusions

References

Part V: Rhythm Disturbances in the Newborn

Chapter 59: Structural, Metabolic, and Genetic Abnormalities Affecting the Neonatal Conduction System

References

Chapter 60: Tachydysrhythmias

Supraventricular Tachycardia

Ventricular Tachydysrhythmias

References

Chapter 61: Bradydysrhythmias

Diagnosis of Abnormal Atrioventricular Conduction

Etiologies of Abnormal Atrioventricular Conduction

Inherited Causes of Bradycardia

Non-Cardiac Causes of Bradycardia

Evaluation

Management

Conclusions

References

Chapter 62: Atrial and Ventricular Ectopies

Premature Atrial Contractions

Clinical Presentation

Evaluation

Management

Premature Ventricular Contractions

Clinical Presentation

Evaluation

Management

References

Part VI: Special Issues in the Newborn

Chapter 63: Balloon Atrial Septostomy

References

Chapter 64: Interventional Therapeutic Procedures in the Newborn

Opening of Atrial Communication

Transcatheter Balloon Dilation of Cardiac Valves

Balloon Angioplasty and/or Stent Placement

PDA Stenting

Transcatheter Vascular Occlusion

Closure of Intracardiac Communications (ASD, VSD)

References

Chapter 65: The Hybrid Procedure

Hybrid Palliation of Hypoplastic Left Heart Syndrome

Creating a Non-Restrictive Atrial Communication

Perventricular Closure of Ventricular Septal Defects

Hybrid Balloon Aortic or Pulmonary Valvuloplasty

References

Chapter 66: Neonatal Cardiac Surgical Procedures

Hypoplastic Left Heart Syndrome (Figure 66.1)

Transposition of the Great Arteries (Figure 66.5)

Systemic to Pulmonary Artery Shunt (Figure 66.9)

Neonatal Repair of Coarctation

Total Anomalous Pulmonary Venous Connection

Interrupted Aortic Arch (Figures 66.20 and 66.21)

Truncus Arteriosus (Figures 66.22, 66.23, 66.24, and 66.25)

Patent Ductus Arteriosus (Figures 66.26 and 66.27)

Chapter 67: Extracorporeal Membrane Oxygenation and Ventricular Assist Devices

Indications for Circulatory Support

Types of Mechanical Circulatory Support Devices

Management During Circulatory Support

Experience with Berlin Heart EXCOR

Conclusions

The Future

References

Chapter 68: Neonatal Cardiac Transplantation

History

Demographics of Transplant

Epidemiology

Post-Transplant Survival

Quality of Life

Immunosuppression

Morbidity

Waitlist Mortality

Benefits

Transplantation and Hypoplastic Left Heart Syndrome

Heart Transplant Technique

Conclusions

References

Chapter 69: Postoperative Care of the Newborn

General Principles

Inotropic Support of the Postoperative Patient

Mechanical Support of the Failing Heart After Surgery

Sedation, Analgesia, and Neuromuscular Blockade

Postoperative Arrhythmias

Special Considerations

Issues on Postoperative Care in the Newborn for Specific Lesions (see also chapters on specific cardiac defects)

Part VII: Neonatal Formulary

Chapter 70: Drugs

Further Reading

Chapter 71: Nutrition

Stress Response to Surgery or Shock

Metabolic Needs in Uncorrected CHD

Feeding

References

Index

End User License Agreement

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Guide

Cover

Table of Contents

Preface

Begin Reading

List of Illustrations

Chapter 1: Cardiac Embryology and Embryopathy

Figure 1.1 The scanning electron micrograph image shows the developing heart of the mouse at E9.5. The heart has been revealed by removing the ventral wall of the pericardial cavity. The ventricular loop extends from the atrioventricular (AV) canal, and supports the outflow tract.

Figure 1.2 The image is prepared using an episcopic dataset from a developing mouse embryo early on embryonic day 11.5. The four-chamber section shows how the atrial appendages are beginning to balloon in parallel fashion from the common atrial chamber, while the apical components of the developing ventricles are ballooning in series from the ventricular loop. The process of ballooning of the apical ventricular components produces the muscular ventricular septum formed between them (star). The AV canal connects predominantly to the developing left ventricle (LV), but already its right wall has provided contiguity between the right atrium (RA) and the developing right ventricle (RV; double-headed white arrow).

Figure 1.3 The scanning electron microscopic image prepared from a

Pitx2c

knock-out mouse shows the atrial chambers, viewed from the ventricular aspect, having cut the heart in its short axis. There is isomerism of the RA appendages.

Figure 1.4 The image is from an episcopic dataset prepared from a mouse at early embryonic day 11.5. A short axis cut has been made through the ventricular loop, which is then viewed from the aspect of the transected apical components. The star shows the developing ventricular septum. The opening between the AV cushions opens exclusively into the cavity of the developing LV. The outflow tract is supported by the developing RV.

Figure 1.5 The image is from an episcopic dataset prepared from a mouse at early embryonic day 11.5. The apical trabecular component of the RV is beginning to balloon from the outlet component of the ventricular loop. As yet, there is no direct communication between the cavities of the RA and the RV, the blood flowing into the developing RV through the embryonic interventricular communication. Already, however, the right wall of the AV canal (double-headed arrow) provides continuity between the RA and RV walls. The outflow tract arises exclusively from the RV, with the proximal outflow cushions already visible within its lumen (stars).

Figure 1.6 The scanning electron micrograph image shows evidence of the initial symmetry of the systemic venoatrial connections at embryonic day 9.5 in the mouse, albeit that the left horn is smaller than the right. The section is taken through the dorsal mesocardium, and shows the pulmonary pit (thick arrow). As yet, there is no formation of the lungs.

Figure 1.7 The scanning electron microscopic image shows the atrial chambers, viewed from the aspect of the removed ventricular chambers, from a developing mouse heart obtained late at E10.5. The dissection shows how the left sinus horn, with its own discrete walls, has become incorporated into the developing left AV junction. Note the secondary atrial foramen.

Figure 1.8 The image is from an episcopic dataset prepared from a human embryo at Carnegie stage 14. It shows the atrial cavities viewed from the ventricular aspect. The left sinus horn has been incorporated in the left AV groove, and the openings of the caval veins are seen within the confines of the venous valves (stars). Note the location of the primary atrial septum, which is growing from the atrial roof.

Figure 1.9 The image is from the same dataset as shown in Figure 1.8, but is cut in the sagittal plane, replicating the long axis parasternal echocardiographic plane. It shows the AV cushions facing one another in the AV canal, and the outflow cushions (stars) extending the full length of the outflow tract. Note also the ventral protrusion from the dorsal wall of the aortic sac. The section also cuts through the solitary pulmonary vein, and its entrance to the developing LA, which at this stage is adjacent to the developing AV junction. The double-headed white arrow shows the sectioned primary atrial septum, which separated the primary (Foramen 1) and secondary (Foramen 2) atrial foramens. Note the discrete walls of the left sinus horn, now incorporated within the left AV junction.

Figure 1.10 The four-chamber section is prepared from an episcopic dataset from a mouse heart at embryonic day 18.5. The mesenchymal cap and vestibular spine have muscularized to form the anteroinferior buttress of the oval fossa (double-headed white arrow). The cranial margin of the fossa, however, is a deep fold between the RA wall and the attachments of the pulmonary veins to the LA. The floor of the oval fossa is formed by the primary atrial septum. Note the discrete walls of the left sinus horn, which in the mouse persists as a left superior caval vein.

Figure 1.11 The four-chamber section is from an episcopic dataset prepared from a mouse heart at embryonic day 11.5. It shows the building blocks of the atrial septum. The primary septum has broken away from the atrial roof to form the secondary foramen. The space between the mesenchymal cap on its leading edge and the inferior AV cushion is the primary atrial foramen. Note the vestibular spine at the leading edge of the valves guarding the systemic venous sinus of the RA.

Figure 1.12 The four-chamber section is from an episcopic dataset prepared from a mouse heart at embryonic day 13.5. The mesenchymal cap on the atrial septum has fused with the AV cushions to close the primary atrial foramen. The section is cut more dorsally, and shows how the vestibular spine has reinforced the right side of the area of fusion. The spine is beginning to muscularize to form the anteroinferior buttress of the oval fossa (see Figure 1.10).

Figure 1.13 The four-chamber section is from an episcopic dataset prepared from a mouse heart at embryonic day 12.5. It shows the vestibular spine growing from the site of the right pulmonary ridge. The arrow shows the connection with the pharyngeal mesenchyme. The spine is carrying forward to inferior zone of apposition of the venous valves that guard the systemic venous sinus. Note the left superior caval vein, derived from the left sinus horn, entering the left AV junction.

Figure 1.14 The four-chamber section is from an episcopic dataset prepared from a

Tbx1

null mouse at embryonic day 12.5. The mouse has an AV septal defect, with this section showing the ostium primum defect. There is total lack of formation of the vestibular spine. Note the hypoplastic nature of the right pulmonary ridge.

Figure 1.15 The image is the same as that used for Figure 1.2, and comes from a developing mouse embryo early on embryonic day 11.5. It is re-labeled to show how, at this early stage, the AV canal connects almost exclusively with the cavity of the developing LV (bracket). The blood then enters the developing RV through the embryonic interventricular communication (double-headed white arrow), which is bounded caudally by the developing muscular ventricular septum (star), and cranially by the right margin of the inner heart curvature (white curve).

Figure 1.16 The image is a frontal section through an episcopic dataset prepared from a developing mouse early on E12.5. The AV canal has expanded so that the cavity of the developing RA is now in direct continuity with the cavity of the RV, thus producing the RV inlet. The larger parts of the AV cushions, however, remain committed to the LV. The aortic component of the developing outflow tract, in contrast, remains supported by the developing RV, so that the blood entering the aorta must still pass through the embryonic interventricular communication (white arrow). The star shows the crest of the muscular interventricular septum.

Figure 1.17 The image shows a four-chamber section through the AV junctions later on embryonic day 12.5 in the developing mouse heart. The major AV endocardial cushions have now fused to divide the junction into the tricuspid (TV) and mitral valve (MV) orifices. Lateral cushions have now developed in the newly formed right and left junctions, and will form the mural leaflet of the MV, and the anterosuperior and inferior leaflets of the TV (see Figure 1.18). The star shows the muscularizing vestibular spine and mesenchymal cap, which bind the atrial septum to the surface of the cushions.

Figure 1.18 The image shows a short axis section from an episcopic dataset prepared from an embryonic mouse at day 13.5. The bulk of the fused AV cushions remains within the LV and have fused to form what will become the aortic leaflet of the MV. At this stage, however, the aortic outflow tract remains supported by the RV (star). The right lateral cushion and the rightward margins of the fused AV cushions guard the developing TV orifice.

Figure 1.19 The image showing the short axis of the ventricular mass viewed from the apex is from an episcopic datasets prepared from a mouse at embryonic day 14.5. The aortic root has now been transferred into the LV, interposing between the septum and the MV so that the latter valve now possesses aortic and mural leaflets. The TV is developing its anterosuperior and inferior leaflets, but the septal leaflet has not yet delaminated from the muscular ventricular septum.

Figure 1.20 The episcopic section shows the right side of the developing mouse heart at embryonic day 13.5. The proximal outflow cushions have fused to form the cranial margin of the persisting embryonic interventricular communication (white circle).

Figure 1.21 This episcopic section, in the same plane as Figure 1.20, is from a mouse at embryonic day 14.5. The surface of the fused proximal cushions has muscularized to form the margin of the free-standing infundibular sleeve adjacent to the aortic root.

Figure 1.22 This episcopic section, again from a mouse at embryonic day 14.5, but now in four-chamber projection, shows how the rightward margins of the AV endocardial cushions have fused to close the RV entrance to the subaortic vestibule, thus forming the membranous septum, and completing the process of ventricular septation.

Figure 1.23 The image is from an episcopic dataset prepared from a human embryo at Carnegie stage 13. The cavity of the distal outflow tract becomes continuous with the lumen of the aortic sac at the level of the pericardial reflections (thick arrows). Arising from the aortic sac are the arteries running through the fourth and sixth pharyngeal arches. The fourth arch arteries will become the systemic channels, while the sixth arch arteries will supply the developing pulmonary arteries, so at this stage the dorsal wall of the sac (star) is the putative aortopulmonary septum.

Figure 1.24 The image shows orthogonal sections from an episcopic dataset from a mouse at embryonic day 11.5, with reconstruction of the arteries running through the pharyngeal mesenchyme. At this stage, the arteries of the fourth arch are dominant, and bilaterally symmetrical, as are the arches of the first through third arches, which are diminishing in importance, and those of the sixth arches, which are still developing.

Figure 1.25 The image is a reconstruction made from an episcopic dataset prepared from a mouse at embryonic day 14.5. It shows how, because of regression of the right-sided components of the initially bilaterally symmetrical arteries coursing through the pharyngeal pouches, the system has been transformed into the arch of the aorta and the arterial duct. The seventh intersegmental arteries (stars), however, have still to migrate cranially to become the subclavian arteries. PT, pulmonary trunk.

Figure 1.26 The image is from an episcopic dataset prepared from a developing mouse heart at embryonic day 11.5. It shows a long axis cut through the developing outflow tract. The parietal walls of the aorta and pulmonary trunk have grown into the heart from the second heart field. The distal outflow tract is now undergoing separation by fusion of a ventral intraluminal protrusion from the pharyngeal mesenchyme enclosing the aortic sac (star) with the distal ends of the cushions that extend through the outflow tract.

Figure 1.27 The image is from an episcopic dataset prepared from a developing mouse heart at embryonic day 13.5. It is a frontal section showing the part of the aortic root adjacent to the newly separated pulmonary root. Note the fused mass of the proximal outflow cushions that produced the separation. The bud of the left coronary artery, arising from the aortic trunk distal to the ends of the outflow cushions, which will cavitate to form the aortic valvar leaflets. The bud has joined with the epicardial component of the developing left coronary artery.

Figure 1.28 The image, this time in short axis, and viewed from above, is from an episcopic dataset prepared from a mouse heart at embryonic day 15.5. The origins of the coronary arteries, which were originally formed distal to the junction between the developing sinuses and the intrapericardial aorta (see Figure 1.27), have now been incorporated within the aortic valvar sinuses adjacent to the pulmonary root. Dashed line, transient aortopulmonary foramen.

Chapter 2: Maternal, Familial, and Non-Cardiac Fetal Conditions Affecting the Fetal and Neonatal Heart

Figure 2.1 Neonatal hypertrophic cardiomyopathy associated with maternal diabetes. (a) Parasternal long axis and (b) short-axis images demonstrate asymmetric septal hypertrophy with near obliteration of the left ventricular cavity. The development of hypertrophy is associated with maternal hemoglobin A1C levels >6 g/dL in the latter part of gestation and generally resolves slowly after birth.

Figure 2.2 Maternal autoantibody-associated heart block and cardiomyopathy. (a) M-mode tracing in a 26 weeks' gestation fetus with AV dissociation consistent with third-degree AV block (a, atrial contraction; v, ventricular contraction) in the setting of SSA+ mother. Endocardial fibroelastosis involving the left ventricle is seen (b) by fetal echocardiography, as echo-bright areas of atrial and ventricular endocardium (arrows) and (c) autopsy specimen. Heart block is usually not reversible once it occurs; cardiomyopathy, associated with high rates of death or transplantation in the first year of life, may be treated in utero or in the neonatal period with dexamethasone and intravenous immunoglobulin with improvement in survival [18]. Photo (c) courtesy of Norman H. Silverman, MD, DSc (MED).

Figure 2.3 Constriction of the fetal ductus arteriosus resulting from maternal indomethacin exposure. (a) Short-axis image near the base of the fetal heart showing color Doppler aliasing (arrow) in the ductus with (b) corresponding spectral Doppler signal demonstrating increased velocities in systole (s), early diastole (ed), and late diastole (ld).

Figure 2.4 Left ventricular non-compaction cardiomyopathy. The left ventricular myocardium has a “spongy” appearance (a, arrows) with large non-compacted to compacted layer dimension, which can be demonstrated clearly by application of color Doppler over the area of interest (b). This neonate presented with poor cardiac output and the diagnosis was confirmed on gross and histologic examination of the explanted heart at the time of orthotopic heart transplantation soon after these images were obtained.

Figure 2.5 Multiple cardiac rhabdomyomas in a neonate with tuberous sclerosis. Though rarely obstructive, in this particular case there was moderate obstruction to the left ventricular outflow tract seen by color Doppler from the subcostal coronal image (lower right). These tumors can be detected in the mid-gestation fetus, and often enlarge at an alarming rate through the remainder of gestation, but will regress postnatally.

Figure 2.6 Fetus with large sacrococcygeal teratoma (SCT). (a) Intraoperative photo during fetal resection and debulking of the tumor (T); (b) Preoperative fetal echocardiography demonstrating cardiomegaly and pericardial effusion associated with impending hydrops because of high cardiac output. SCT is a germ cell tumor originating at the coccyx and growing outward and often occupying the fetal pelvis and abdomen that can grow to a substantial and highly vascular mass. SCT can cause high output cardiac failure because of increased preload which is exacerbated by its typically low vascular resistance which can compete with blood flow to placenta. With large SCT, the cardiac findings are ventricular hypertrophy and dilatation, with reduction in systolic function, atrioventricular valve regurgitation with development of hydrops and risk of fetal demise. Fetal surgical intervention or early delivery may be indicated for severe cases.

Figure 2.7 Congenital pulmonary airway malformation (CPAM, formerly congenital cystic adenomatoid malformations or CCAM). (a) Axial image of the thorax, note the extreme rightward shift and compression of the heart and the large mixed microcystic and macrocystic CPAM. (b) Sagittal image of the same fetus, demonstrating flattening of the left diaphragm (arrowheads) and a moderate accumulation of ascites. These lesions are complex histologically and may appear on ultrasound as homogeneous, cystic, or mixed type lesions. Compression of the heart and large veins can lead to impaired cardiac filling and hydrops. Myocardial function may appear preserved yet some studies have demonstrated reduced function over time.

Figure 2.8 Fetal pericardial teratoma (X) with associated pericardial effusion at 26 weeks' gestation. The teratoma arises from the base of the heart near the aorta or right AV groove (here resulting in external compression of the right atrium) and usually receives blood supply from a vascular stalk from the ascending aorta. Though pericardial teratomas and their accompanying pericardial effusions are often well-tolerated, large teratomas can also have a compressive effect and lead to hydrops in the fetus or severe compromise in the newborn necessitating urgent surgery for decompression and/or removal of the tumor. E, effusion; LV, left ventricle; RV, right ventricle.

Chapter 3: The Natural and Unnatural History of Fetal Heart Disease

Figure 3.1 Natural progression of ventricular outflow obstruction (aortic/pulmonary stenosis).

Figure 3.2 Progression of aortic stenosis in a fetus. The earlier the outflow obstruction develops in the fetus, the higher the likelihood of progression of left ventricle (LV) hypoplasia. (a) At 21 weeks, the LV has normal size and systolic function. (b) The aortic valve is thickened (arrow) causing LV outflow tract obstruction. (c) Color Doppler demonstrating turbulence across the LV outflow tract. (d) The peak gradient was 46 mmHg. (e) As the obstruction increases, the LV becomes progressively more dilated with reduced systolic function; there can be reversal of flow across the foramen ovale and aortic arch. To change the natural history of the disease and potential development of hypoplastic left heart syndrome, the patient underwent closed fetal cardiac intervention with balloon valvuloplasty of the aortic valve. (f) After the procedure, the LV is normal in size and function with normal right to left shunting across the patent foramen ovale. (g) Postnatally, the LV was normal in size and only aortic balloon valvuloplasty was needed. LV, left ventricle, Ao, ascending aorta.

Figure 3.3 Echocardiographic imaging in a fetus with severe aortic valve stenosis resulting in hydrops fetalis. Unlike the previous case, at 30 weeks, severe mitral regurgitation (MR) was present. In the setting of outflow tract obstruction, the usual evolution is progressive ventricular hypoplasia. However, when significant atrioventricular valve insufficiency develops, the ventricle undergoes significant dilatation. (a) Four-chamber view shows severe left ventricle (LV) dilatation. There is severely decreased systolic function, and left atrium (LA) dilatation which compresses the right atrium (RA). (b) Severe mitral regurgitation. (c) Development of significant ascites and hydrops.

Figure 3.4 Progression of right ventricular outflow tract (RVOT) obstruction. (a) At 20 weeks, the pulmonary valve is doming and thickened. The right ventricle (RV) is mildly hypertrophied with normal systolic function. (b) Color flow mapping revealed turbulence at the level of the valve. (c) Peak gradient is 34 mmHg. (d) At 24 weeks, there is moderate RV hypertrophy and hypoplasia, with severe tricuspid regurgitation (TR) by color flow Doppler (e), predicting a suprasystemic right ventricular pressure (RVP). PA, pulmonary artery.

Figure 3.5 Natural history and progression in the fetus with significant atrioventricular valve regurgitation (e.g., Ebstein anomaly). LV, left ventricle; RV, right ventricle; RVOT, right ventricular outflow tract.

Figure 3.6 Severe Ebstein anomaly. (a,b) Severe tricuspid regurgitation with severe right atrial (RA) and right ventricular (RV) dilatation. This progressed in pregnancy and (c) resulted in significant increase in the cardiothoracic ratio and compression of the left ventricle (LV). (d) Decrease in forward flow through the RV outflow tract results in functional pulmonary atresia with reversal of flow in the ductus arteriosus (arrow).

Figure 3.7 Tetralogy of Fallot with absent pulmonary valve syndrome. (a) Anterior malalignment ventricular septal defect (VSD; arrow) and free pulmonary regurgitation (color) cause progressive dilatation of the proximal pulmonary arteries (PA) which can result in significant compression on the tracheal–bronchial tree and esophagus. (b) Free pulmonary regurgitation shown by Doppler across the RV outflow tract.

Figure 3.8 D-transposition of the great arteries. Progression of events leading to significant postnatal hypoxemia and need for urgent balloon atrial septostomy. LA, left atrium; PDA, patent ductus arteriosus; PFO, patent foramen ovale.

Figure 3.9 D-transposition of the great arteries. The progressive increase in the left atria blood flow results in increased pressure and causes the atrial septum to oscillate between (b) the right and (a) left atrium. This can be predictive of significant hypoxemia after birth requiring urgent balloon septostomy. LA, left atrium; RA, right atrium.

Figure 3.10 Ventricular septal defect (VSD). (a,b) Small mid-muscular VSD (arrow). Such defects often close spontaneously, up to one-third in utero. (c) Large muscular VSD in the apical muscular septum (arrow). This size of defect is less likely to close in utero and may require intervention postnatally. (d) Large posterior malalignment VSD with an overriding aorta (Ao). These defects do not close spontaneously and can be associated with chromosomal abnormalities.

Figure 3.11 Fetal arrhythmias. (a) Atrial flutter; (b) supraventricular tachycardia. Fetal tachyarrhythmias, which usually develop in the late second or third trimesters, can progress and lead to significant fetal compromise necessitating early recognition and transplancental therapy. (c) Similarly, complete heart block, either associated with structural heart disease or more commonly with maternal autoantibodies, can lead to significant cardiovascular compromise.

Figure 3.12 Cardiac tumors. (a,b) Pericardial teratoma (arrow) progresses in size and results in severe pericardial effusion and hydrops fetalis. (c) Rhabdomyomas tend to be multiple (arrows) and frequently increase in size potentially leading to inflow or outflow obstruction before regressing. (d,e) Large rhabdomyoma (arrows) occupying most of the LV and obstructing flow resulting across the LV outflow tract and resulting in near hypoplastic left heart syndrome.

Chapter 5: The Transitional and Neonatal Heart and Cardiovascular System

Figure 5.1 Two-dimensional subcostal view demonstrating small predominantly closed foramen ovale.

Figure 5.2 Color Doppler subcostal view demonstrating small amount of left to right shunting across a predominantly closed foramen ovale.

Figure 5.3 Color Doppler parasternal short axis view of a patent ductus arteriosus (PDA).

Figure 5.4 Four-chamber view showing dominance of right-sided chambers.

Figure 5.5 Parasternal short axis view showing both right ventricular (RV) volume and septal flattening in a normal newborn.

Figure 5.6 Parasternal long axis medially angulated tricuspid regurgitation Doppler pattern demonstrating elevated right ventricle and pulmonary artery systolic pressure.

Figure 5.7 Cropped parasternal two-dimensional image of main pulmonary artery (MPA) and pulmonary annulus slightly larger than aorta.

Figure 5.8 High parasternal short axis color Doppler image of flow acceleration at the origin of the branch pulmonary arteries.

Figure 5.9 Summary timeline of key neonatal cardiovascular changes. DV, ductus venosus; MPA, main pulmonary artery; PBPS, peripheral branch pulmonary stenosis; PDA, patent ductus arteriosus; PFO, patent foramen ovale; RV, right ventricle.

Chapter 7: The Cyanotic Newborn

Figure 7.1 Recommended pulse oximetry screening for infants after 24 hours in the hospital and prior to hospital discharge. Any infant with a positive screen should have a diagnostic echocardiogram, which would involve an echocardiogram within the hospital or birthing center, transport to another institution for the procedure, or use of telemedicine for remote evaluation. The infant's pediatrician should be notified immediately and the infant might need to be seen by a cardiologist for follow-up. ox, oximetry; RH, right hand.

Chapter 10: The Dysmorphic Newborn

Figure 10.1 Noonan syndrome.

Figure 10.2 Trisomy 13: (a) cutis aplasia; (b) clouded cornea.

Figure 10.3 Trisomy 21/Down syndrome: (a) upslanting palpebral fissures and epicanthal folds; (b) low nasal bridge and prominent tongue; (c) single palmar crease.

Figure 10.4 22q11 deletion syndrome: (a–c) periorbital fullness, prominent nasal root, bulbous nasal tip, and pinched alae.

Figure 10.5 Williams syndrome.

Figure 10.6 CHARGE syndrome ears. Deficient lobes and “clipped-off” helices.

Figure 10.7 Upper extremity, hand, and finger anomalies: (a) hypoplastic thumb and wrist anomalies of Holt–Oram syndrome; (b) radial ray anomalies with absent radius and thumb in VACTERL syndrome; (c) polydactyly of the hand in trisomy 18; (d) overlapping fingers and clenched fists of trisomies 18 and 13.

Figure 10.8 Foot and toe anomalies: (a) polydactyly of the foot; (b) dorsal lymphedema of the feet in Turner syndrome; (c) rocker-bottom feet of trisomies 18 and 13.

Figure 10.9 Cleft lip and palate in trisomy 13.

Chapter 11: Chest Roentgenogram

Figure 11.1 Frontal radiograph of the chest in a patient with a ventricular septal defect (VSD) shows increased pulmonary vascularity; prominent pulmonary vessels seen bilaterally.

Figure 11.2 Frontal radiograph of the chest in a patient with obstructed total anomalous venous return shows increased indistinctness of the pulmonary vessels from diffuse pulmonary edema and fluid seen in the right costophrenic angle and horizontal fissure (black arrows).

Figure 11.3 Frontal radiograph of the chest in a patient with Ebstein anomaly shows decreased pulmonary vascularity and marked cardiomegaly. Notice how clear the lungs are with very few vessels seen (arrow).

Figure 11.4 Frontal radiograph of the chest in a patient with tetralogy of Fallot shows decreased pulmonary vascularity and an upturned apex of the heart (arrow). This is often referred to as a “boot”-shaped heart.

Figure 11.5 (a) Frontal and (b) lateral radiographs of the chest in an asymptomatic patient shows apparent cardiomegaly on the frontal view with a normal heart size on the lateral view. Thymic tissue in infants may make the cardiac silhouette appear enlarged.

Figure 11.6 Both patients show the stomach to be on the right. (a) The patient has the cardiac apex on the right consistent with situs inversus; (b) the patient has the cardiac apex on the left consistent with situs ambiguous.

Figure 11.7 Frontal radiograph of the chest in a patient with repair of a prior tracheoesophageal fistula. Notice that there is cardiomegaly in this patient with a large atrial septal defect (ASD) and VSD. Also notice the hemivertebral body at L2 and the absent right radius. Patients with congenital disease heart often have associated anomalies that follow the VACTERL mnemonic.

Figure 11.8 Lateral radiograph of the chest in a patient with known Down syndrome. Notice the hypersegmented manubrium (arrow) which is often seen in patients with Down syndrome on lateral view of the chest.

Figure 11.9 Frontal radiograph of the chest in an asymptomatic patient shows a curvilinear vein coursing anteromedically towards the inferior vena cava (black arrow). This was a partial anomalous venous return. Also notice the rightward mediastinal shift caused by hypoplasia of the right lung consistent with hypogenetic lung syndrome or scimitar syndrome.

Figure 11.10 Frontal radiograph of the chest in a patient with high blood pressure in the upper extremity shows a classic “Figure 3” sign from dilatation of the left subclavian artery (white arrow) and post-stenotic dilatation of the proximal descending thoracic aorta (black arrow) in a patient with coarctation. Subtle sclerosis and undulation of the inferior surface of the ribs is seen consistent with rib notching from prominent arterial collaterals (arrowhead).

Figure 11.11 Frontal radiograph of the chest in a patient with supracardiac type total anomalous pulmonary venous return (TAPVR). Notice the prominent superior mediastinum from the anomalous venous drainage forming a snowman-like appearance of the mediastinum.

Figure 11.12 Frontal radiograph of the chest in a patient with dextro-transposition of the great arteries shows a globular or egg-shaped heart with a narrow mediastinum. The “egg on a string.”

Figure 11.13 Frontal radiograph of the chest in a patient with tetralogy of Fallot shows decreased pulmonary vascularity and an upturned apex of the heart (arrow). This is often referred to as a “boot”-shaped heart.

Figure 11.14 Frontal radiograph of the chest showing a left upper extremity PICC line descending along the left mediastinum in a left superior vena cava (arrow).

Figure 11.15 Frontal radiograph of the chest showing a large pneumopericardium in a patient after drainage of a large effusion.

Chapter 12: Electrocardiogram

Figure 12.1 Orientation of leads of the electrocardiogram (ECG). Forces directed towards the leads are positive or upright deflections while forces moving away from the leads are negative or downward deflections.

Figure 12.2 ECG tracing with P wave, QRS complex, T and U waves.

Figure 12.3 Long QT syndrome with corrected QT interval of 550 ms. Note that the T wave ends just before the onset of the next P wave.

Figure 12.4 (a) Right atrial enlargement with P waves more than three boxes tall; (b) left atrial enlargement with P waves more than two boxes wide.

Figure 12.5 Wolff–Parkinson–White pattern with shortened PR interval and delta wave (slurring of the initial upstroke of the QRS complex).

Figure 12.6 Anomalous left coronary artery with anterior infarction pattern (Q waves more than ½ box and ST segment changes in leads I and aVF. Note the abnormal Q waves and ST segment changes in lateral leads (V4–V6).

Figure 12.7 Loss of distinction between QRS complex and T wave (sine wave pattern) characteristic of hyperkalemia and severe acidosis or hypoxemia.

Chapter 13: Echocardiogram

Figure 13.1 There are four main pediatric windows for optimal imaging: subcostal, apical, parasternal, and suprasternal views.

Figure 13.2 Subcostal transverse abdominal views in a patient showing the normal position of the liver on the right and the stomach on the left. The inferior vena cava (IVC) sits rightward and anterior to the aorta which is located just leftward of the spine.

Figure 13.3 (a) Subcostal frontal views starting posteriorly allowing visualization of the atrial septum. In this case there is a small patent foramen ovale. Both the left atrium (LA) and right atrium (RA) are well seen as is the left lower pulmonary vein (LLPV) and right superior vena cava (RSVC). The right pulmonary artery (RPA) is identified posterior to the RSVC. (b) Subcostal frontal image sweeping anterior demonstrating the left ventricle (LV), mitral valve (MV), and outflow tract. The aortic valve (AV) and ascending aorta (AsAo) are well visualized. Just leftward of the aorta is the main pulmonary artery (MPA). The ventricular septum is also well seen in this image.

Figure 13.4 (a) Apical view demonstrating all four chambers: the left atrium (LA), right atrium (RA), left ventricle (LV), and right ventricle (RV). Both the mitral valve (MV) and tricuspid valve (TV) are well seen. (b) Tilting the probe anterior from the four-chamber view allows for visualization of left ventricular outflow tract and aortic valve (AV). The left circumflex coronary artery (LCx) is seen running adjacent to the mitral valve. (c) Apical four-chamber view with color flow placed over the mitral valve demonstrating a significant jet of mitral regurgitation (MR) directed back into the left atrium. The bright blue and yellow colors produced by the MR jet are related to the direction of flow and high velocity of the blood as it regurgitates back into left atrium (LA).

Figure 13.5 Parasternal long axis imaging of the left ventricular outflow tract allowing for visualization of the aortic valve (AV), aortic root (AoR), sinotubular junction (STJ), and ascending aorta (AsAo). Assessments of the mitral valve and left ventricle (LV) can also be made from this view.

Figure 13.6 A color compare image taken in a parasternal short-axis view demonstrating the right ventricular outflow tract, main pulmonary artery (MPA), and both left (LPA) and right pulmonary arteries (RPA). Two-dimensional view on the left and color flow of the same image on the right. The right atrium (RA), right ventricle (RV), and aortic valve (AV) are also identified in this view.

Figure 13.7 From suprasternal notch the “candy cane” view of the aortic arch demonstrates the ascending (AAo), transverse (TrA) and descending aorta (DAo) along with the head and next branches including the left innominate artery, left common carotid (LCC), and the left subclavian artery (LSCA). The aortic isthmus and descending aorta (DAo) are visualized. The left pulmonary artery is seen coursing underneath the aorta and the left innominate vein (LIV) runs superior and anterior to the aortic arch.

Figure 13.8 Parasternal imaging of the left atrium (LA) in the “crab view” provides identification of all four pulmonary veins. The left upper (LUPV), left lower (LLPV), right upper (RUPV) and right lower (RLPV) pulmonary vein are all identified by color flow assessment. The aorta (Ao) and pulmonary artery (PA) are visualized superior to the LA.

Chapter 14: Cardiac Catheterization and Angiocardiography

Figure 14.1 Various types of patent ductus arteriosus (PDA) using the classification of Krichenko

et al

. [2]. AO, aorta; PA, pulmonary artery.

Figure 14.2 Various morphologies of PDA. (a) Silent ductus; (b–c) small to large type A ductus; (e) complex type D ductus; (f) serpiginous ductus often seen in tricuspid or pulmonary atresia.

Figure 14.3 Patient with critical pulmonary valve stenosis. (a) Pulmonary valve jet (arrow) from right ventricle into main pulmonary artery before valvuloplasty; (b) improved flow across valve post valvuloplasty; (c) waist on balloon depicts the location of the valvar stenosis; (d) resolution of waist indicating successful valvuloplasty. MPA, main pulmonary artery; RV, right ventricle.

Figure 14.4 (a) Severe valvar aortic stenosis. The arrow depicts negative contrast (area of non-contrast opacified blood entering the contrast opacified aortic root) indicating the effective orifice of the valve. (b) Image performed post valvuloplasty reveals mild aortic valve insufficiency (arrowhead).

Figure 14.5 Various forms of coarctation of the aorta in the neonatal period.

Figure 14.6 (a) A: Balloon angiographic catheter positioned in the right ventricle. B: level of aortic valve. C: right ventricular outflow tract. The asterisk marks the same spot in both panels (just past level of pulmonary valve annulus) and is where the tip of the catheter is in (b). D: ascending aorta. E: 4th aortic branch – anomalous right subclavian artery originating from descending aorta. F: 1st aortic branch – right common carotid artery. G: 2nd aortic branch – left common carotid artery. H: 3rd aortic branch – origin of left subclavian artery, which branches medially into: I: the single large MAPCA which descends caudally and branches to supply both right and left pulmonary circulations. (b) Having crossed the level of the atretic pulmonary valve, the severely hypoplastic native pulmonary arteries are shown to be confluent but measuring less than 2 mm in diameter.

Figure 14.7 Lateral projection angiogram with 60° left anterior oblique (LAO) and 30° cranial angulation. Shows a smooth walled, posterior left ventricle (LV) communicating with the pulmonary arteries (MPA). The right ventricle (RV), which is not completely filled with contrast, communicates with the transposed and anterior aorta (AO). There is a ventricular septal defect (VSD) seen as well, which is sometimes present in this lesion.

Figure 14.8 (a) A severely hypoplastic left ventricle following Norwood procedure. The area traced by the white line represents that which a normal-sized left ventricle might occupy in a normal heart. (b,c) A Blalock–Taussig shunt (BTS) originating from the region of the right common carotid artery and right subclavian artery (RSCA) and inserting into the region of the main pulmonary artery (MPA). LPA, left pulmonary artery; RPA, right pulmonary artery. (d,e) A comparable Sano shunt originating from the right ventricle (RV) and inserting into the MPA. (f) An alternative hybrid procedure with placement of bands on the pulmonary arteries to restrict pulmonary blood flow (arrow).

Figure 14.9 (a) A supracardiac total anomalous pulmonary venous connection (TAPVC). In this example, drainage of the pulmonary veins is to the vertical vein (VV), left innominate vein (LINNV), superior vena cava (SVC), and finally to the right atrium. (b) An infra-diaphragmatic TAPVC where drainage is via the common pulmonary vein (CPV), below the diaphragm, to the portal venous (PV) system, and finally to the right atrium via the inferior vena cava. Note the level of the diaphragm shown in the two examples. Solid arrows represent direction of flow through the connecting vein. LPV, left pulmonary vein; RHB, right heart border; RPV, right pulmonary vein.

Figure 14.10 Three-dimensional rotational reconstruction allows the operator (and surgeon) to appreciate the interrelationship of the three collateral arteries (in this case, MAPCAs) which was not seen with the initial standard angiogram. Col, collateral; PA, pulmonary artery.

Chapter 15: Computed Tomography

Figure 15.1 Retrospective ECG-gated scan. The X-ray beam (in blue) is on through multiple cardiac cycles.

Figure 15.2 Prospective ECG-gated scan. The X-ray beam (in blue) is not on during the entire cardiac cycle. In this case enough padding was used to cover 50–90% of the cardiac cycle. Notice the rapid heart rate of 137 bpm. The X-ray beam is triggered by the timing of the cardiac cycle.

Figure 15.3 Posterior projection from a 3D color-coded cardiac CT in a patient with mixed type total anomalous pulmonary venous return. The entire right-sided pulmonary veins and left lower pulmonary vein (pink) drain through a common channel (B) to a dilated coronary sinus (A) to the right atrium (C). The left upper pulmonary vein (G) drains to the left brachiocephalic vein (E). The inferior vena cava (IVC) (H) and superior vena cava (SVC) (F) are shown.

Figure 15.4 Axial maximum intensity projection (MIP) and frontal color-coded 3D images from a cardiac CT shows a single left coronary artery (A) arising from the left coronary sinus with the circumflex (C), left anterior descending (LAD) (B), and right coronary (D) arteries all arising from the left coronary artery. Notice that the right coronary crosses in front of the narrowed right ventricular outflow tract (E). The surgeon would need to be notified of this finding before surgery to plan the best approach.

Figure 15.5 (a) An axial and (b,c) two volumetric images from a cardiac CT in an infant with an obstructive cardiomyopathy shows marked thickening of the muscular ventricular septum and left ventricle. The post-processed functional analysis shows a hyperdynamic ejection fraction measuring 71.3%. Notice the gross volume measurements: end systolic (ES) volume = 4.3 mL, end diastolic (ED) volume = 15 mL, and stroke volume = 10.7 mL. Indexed volumes may be obtained by dividing the gross volume by the body surface area.

Figure 15.6 Volume rendered images of the pulmonary vasculature showing the gross volumes for the right and left pulmonary arteries in an infant where the patent ductus arteriosus (PDA) is inserted into the left pulmonary artery. The volume percentage on the right = 2.751/6.245 = 44/%. The volume percentage on the left = 3.494/6.245 = 56%.

Figure 15.7 A frontal and posterior projection from a 3D color-coded cardiac CT in a patient with transposed double outlet right ventricle. Notice that the right ventricle (purple) gives rise to the aorta (red) and contributes to the main pulmonary artery (blue). A subpulmonic ventral septal defect (VSD) (A) is shown. The descending aortic arch is hypoplastic (E). A PDA (green) is widely patent and needed to get enough blood to the descending aorta. An accessory LAD (B) originates from the right coronary sinus adjacent to the origin of the right coronary artery (D). The LAD (C) originates from the left coronary artery.

Figure 15.8 Posterior view of a resin 3D color-coded model in a patient with tetralogy of Fallot. Notice the green aortopulmonary collaterals arising from the descending aorta and right brachiocephalic artery (red).

Chapter 16: Magnetic Resonance Imaging

Figure 16.1 Short axis cine steady state free precession (SSFP) images in a child with an unbalanced atrioventricular septal defect using (a) multiple signal averages and (b) respiratory triggering; both images display similar image quality. LV, left ventricle; RV, right ventricle.

Figure 16.2 Respiratory triggered, spin-echo echoplanar imaging in the paracoronal plane in a child with heterotaxy syndrome. Note the symmetric trachea-bronchial pattern showing bilateral right-sided (eparterial) bronchi.

Figure 16.3 Three-dimensional (3D) volume rendered image from a postoperative contrast enhanced MRA of the chest demonstrating a child with heterotaxy syndrome and history of obstructed supracardiac total anomalous pulmonary venous return. The right and left lower and the right upper pulmonary veins now return to a posterior confluence whose anastomosis to the atrium is now severely stenotic (*) while the left upper pulmonary vein drains to the left superior vena cava. LUPV, left upper pulmonary vein.

Figure 16.4 3D volume rendered image of a child with complex coarctation of the aorta with transverse arch hypoplasia.

Figure 16.5 Spin-echo echoplanar imaging in the paracoronal oblique plane in a child with a double aortic arch showing airway narrowing (*) at the level of the double arch. LAA, left aortic arch; RAA, right aortic arch.

Figure 16.6 Maximum intensity projection image from a contrast enhanced MRA of the chest in a child with heterotaxy syndrome demonstrating systemic venous anomalies including an inferior vena cava that ascends on the right and then enters into the left-sided atrium with the hepatic veins and bilateral superior vena cava to the ipsilateral atria. IVC, inferior vena cava; LHV, left hepatic vein; LSVC, left superior vena cava; RSVC, right superior vena cava.

Figure 16.7 Steady state free precession image demonstrating (a) the left ventricular pulmonary outflow tract, (b) the right ventricular systemic outflow tract, and (c) a computational model of the blood pool demonstrating the complex ventricular septal defects and relationship to the semilunar valves in a child with d-transposition of the great arteries and VSD. AoV, aortic valve; LA, left atrium; LV, left ventricle; PV, pulmonary valve; RA, right atrium; RV, right ventricle; VSD, ventricular septal defect.

Figure 16.8 3D volume rendered image of a child with hypoplastic left heart syndrome, status post Norwood procedure using a Blalock–Taussig shunt, now with severe stenosis of the proximal left pulmonary artery just leftward of the insertion of the shunt. BTS, Blalock–Taussig shunt; LPA, left pulmonary artery; RPA, right pulmonary artery.

Figure 16.9 T1-weighted imaging in the four-chamber geometry of a child with a large rhabdomyoma (*) of the interventricular septum. LV, left ventricle; RV, right ventricle.

Chapter 17: Electrophysiologic Testing, Transesophageal Pacing and Pacemakers

Figure 17.1 Epicardial pacing system. A bipolar lead delivers energy to pace heart between two electrodes that is sewn directly onto the epicardial surface of the heart. The leads are then tunneled to the generator (also known as the “can”) in the abdomen which contains the computer and battery for the pacemaker.

Figure 17.2 Epicardial implantable cardioverter-defibrillator (ICD) system. A standard bipolar pace sense lead is placed on the epicardial surface. A shocking coil (*) is then placed in the pericardial sac and the leads are tunneled to an ICD generator placed in the abdomen. The pacing lead is able to recognize arrhythmia and the generator can deliver a defibrillation shock to the coil to convert potentially fatal ventricular arrhythmias. (Note: The use of a coil in the pericardial sac is an off-label use.)

Chapter 18: Total Anomalous Pulmonary Venous Connection

Figure 18.1 Variants of total anomalous pulmonary venous connection (TAPVC). (a) Supracardiac: both right (RPV) and left (LPV) pulmonary veins join a common pulmonary venous confluence behind the heart, which drains via a vertical vein to the undersurface of the left innominate vein, and thence to the right atrium. (b) Cardiac: the pulmonary venous confluence connects to the coronary sinus (CS), and thence to the right atrium via the coronary sinus ostium. (c) Infradiaphragmatic: the pulmonary venous confluence drains inferiorly via a vertical vein to the portal vein (PV) or hepatic veins (HV) and thence to the right atrium. IVC, inferior vena cava; SMV, superior mesenteric vein; SV, splenic vein. (d) Mixed connections: left pulmonary veins drain to the left innominate vein (LIV), and right pulmonary veins to the coronary sinus in this example.

Source

: Brown 2009 [9]. Reproduced with permission of John Wiley & Sons.

Figure 18.2 Parasternal short axis echocardiogram view with color Doppler flow mapping in this patient with supracardiac TAPVC to the left innominate vein (LIV) shows the pulmonary venous confluence (PVC) which drains via the vertical vein (VV) toward the leftward aspect of the innominate vein. SVC, superior vena cava.

Figure 18.3 Anterior and posterior views of volume-rendered cardiac magnetic resonance angiogram in a 50-year-old man with uncorrected supracardiac TAPVC to the left innominate vein.The right ventricle was severely dilated (end-diastolic volume index 216 mL/m

2

) and the pulmonary-to-systemic flow ratio measured 3.9. Succesful surgical repair was performed after cardiac catheterization confirmed mildly elevated pulmonary artery pressure and resistance (2.1 Wood units).

Figure 18.4 Cardiac TAPVC. Subxiphoid short axis two-dimensional echocardiogram view in a patient with TAPVC to the coronary sinus shows the pulmonary venous confluence (PVC) draining via a communicating vein to the severely dilated coronary sinus (CS). Note the relative hypoplasia of the left atrium and the obligate open position of the patent foramen ovale (arrow) which allows filling of the left heart. RA, right atrium.

Figure 18.5 Posterior view of volume-rendered cardiac magnetic resonance angiogram in a 2-day-old infant girl with infradiaphragmatic TAPVC to the portal vein. The individual left and right pulmonary veins (LPVs and RPVs, respectively) join a vertical confluence (VV) that courses posterior and inferior to the left atrium (LA) before joining the portal vein. Pulmonary venous blood then returns to the right atrium through the inferior vena cava (IVC).

Figure 18.6 Infradiaphragmatic TAPVC. The subxiphoid short axis echocardiogram view in this patient with infradiaphragmatic TAPVC with two-dimensional and color Doppler flow mapping shows the vertical vein (VV) descending just below the level of the diaphragm, and then coursing anteriorly to drain directly into the inferior vena cava (IVC). Note the narrowing of the venous connection to the IVC (arrow), with color Doppler flow acceleration at the connection to the IVC.

Figure 18.7 Mixed TAPVC. Posterior view of volume-rendered cardiac magnetic resonance angiogram in a 3-day-old infant girl with mixed-type TAPVC. The left upper (LUPV) and lingular pulmonary veins join a semihorizontal vein (arrow) that drains into the coronary sinus (CS). The left lower (LLPV) and both right upper and lower (RUPV and RLPV, respectively) join a vertical vein (VV) that courses infradiaphragmatically and drains into the inferior vena cava (IVC) through the portal vein.

Figure 18.8 Anomalous pulmonary venous drainage with malposition of septum primum. (a) Mild leftward malposition with normal pulmonary venous connections results in anomalous drainage of the right upper and lower pulmonary veins to the right atrium. (b) With more severe malposition of septum primum, drainage of all four pulmonary veins is directed anomalously into the right atrium. Note the absence of a well-developed septum secundum; this is common in patients with the polysplenia forms of heterotaxy syndrome. LA, left atrium; LPV, left pulmonary veins; RA, right atrium; RPV, right pulmonary veins.

Source

: Brown 2009 [9]. Reproduced with permission of John Wiley & Sons.

Figure 18.9 Anomalous pulmonary venous drainage. Apical two-dimensional echocardiogram image in a patient with severe leftward malposition of septum primum shows the lack of septum secundum superiorly, normal pulmonary venous connections to the posterior aspect of the left atrium (LA), and leftward malposition of septum primum (arrow), resulting in drainage of all pulmonary veins into the right atrium (RA). PV, pulmonary veins; RV, right ventricle.

Chapter 19: Other Anomalies of Pulmonary and Systemic Venous Connections

Figure 19.1 Supracardiac abnormal pulmonary drainage. Echocardiographic suprasternal views: (a) connection of the vertical vein (V) to the left innominate vein (I) forming the top part of a “snowman” figure; (b) two pulmonary veins (pv) connected to the vertical vein (V). The flow is away from the heart. The left pulmonary artery (P) is to the right.

Figure 19.2 Scimitar syndrome. CT images: (a) hypoplastic right lung and dextroposition of the heart; (b) a volume-rendered angiogram. A small right pulmonary vein connects to the IVC–right atrial junction.

Figure 19.3 Echocardiographic image of the left superior vena cava (SVC) in a patient with L-transposition and a ventricular septal defect (VSD). The left SVC (L) connects to the dilated coronary sinus (CS).

Figure 19.4 (a) Suprasternal echocardiographic image of the left innominate vein (I) coursing inferior to the aorta (A) and connecting to the right superior vena cava (S). (b) Right-sided color Doppler panel shows blob flow in the abovementioned vessels as well as some turbulence in the left pulmonary artery (P).

Figure 19.5 (a) Two-dimensional echocardiography showing the persistent left SVC draining into the left atrium. (b) Computed tomography scan showing bilateral superior venae cavae without the left innominate vein. (c) Three-dimensional CT scan reconstruction showing the absence of communication between the bilateral SVCs. (d) Angiography confirming direct connection of the left SVC to the roof of the left atrium. Ao: aorta; LA: left atrium; LSVC, left superior vena cava; RSVC: right superior vena cava. Source: Tampere

et al.

2012 [19]. Copyright (C) 2012 Elsevier Masson SAS. All rights reserved. Reprinted with permission.

Figure 19.6 Two-dimensional echocardiography. (a) Right superior vena cava (RSVC) connected to the left atrium (LA). (b) Left-to-right shunting across the non-restrictive secundum atrial septal defect and flow from the right pulmonary veins (pv) into the RSVC. Source: Vassallo

et al.

2006 [13]. Reproduced with permission of John Wiley & Sons.

Figure 19.7 Two-dimensional echocardiography of interrupted IVC with azygos continuation in a patient with heterotaxy. (a) The hepatic vein (H) entering the right atrium directly. (b) The azygos vein (Az) entering the right SVC.

Figure 19.8 X-ray of a newborn with persistent right umbilical vein. The catheter in the umbilical artery courses in a normal fashion via the right iliac artery up into the descending aorta with the tip at the level of Th3 (arrow). Catheter inserted in the umbilical vein goes towards the right costophrenic angle ad turns with the tip in a medial direction (arrowhead). Source: Nikstad and Smevik 2004 [22]. Copyright @ 2004 Nakstad and Smevik. Reprinted with permission.

Chapter 20: Anomalies of Atrial Septation