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Congenital Heart Disease, 1st Edition. A Clinical, Pathological, Embryological, and Segmental Analysis

ISBN: 9781560533689
ISBN: 9781560533689
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Authored by the originator of the standard nomenclature for this spectrum of disorders, Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis discusses the history, anatomic features, and physiologic consequences of CHD—in one authoritative resource. The Van Praagh approach to the segmental classification of CHD, developed and implemented by Dr. Richard Van Praagh in the 1960s at Boston Children’s Hospital, remains widely used today, facilitating communication among radiologists, cardiologists, surgeons, and pediatricians who are involved in the diagnosis, characterization, and management of this disease. This unique atlas offers complete coverage of the ubiquitous Van Praagh “language” of CHD, including the signs, symptoms, and clinical manifestations of malpositioned, malformed, or absent cardiovascular chambers, vessels, and valves using traditional as well as state-of-the-art technology.
Key Features
  • Based upon the systematic, widely accepted Van Praagh system of three-part notation used to succinctly describe the visceroatrial situs, the orientation of the ventricular loop, and the position and relation of the great vessels.
  • Demonstrates how the Van Praagh approach facilitates interpreting and reporting findings through cardiac imaging with CT, MR, and ultrasonography, including fetal cardiac imaging.
  • Presents the pathologic anatomy that pediatric and adult cardiologists, radiologists, and echocardiographers need to understand in order to make accurate diagnoses in complex congenital heart disease; as well as the pathologic anatomy that interventionists, pediatric cardiac surgeons, and adult congenital heart surgeons need to know in order to manage their patients successfully.
  • Features more than 550 high-quality images to help you visualize and recognize malformations.
  • Shares the knowledge and expertise of a world-renowned authority on congenital heart disease—a master teacher and the originator of the Van Praagh segmental classification system.
  • Explores the synergy between the various disciplines who manage patient care, including surgeons, radiologists, cardiologists, pathologists, and pediatricians.
  • Enhanced eBook version included with purchase. Your enhanced eBook allows you to access all of the text, figures, and references from the book on a variety of devices.
Author Information
By Richard Van Praagh, MD, Professor Emeritus of Cardiovascular Pathology, Boston Children’s Hospital, Harvard University School of Medicine, Boston, Massachusetts

Περιεχόμενα

  • Cover image
  • Title page
  • Table of Contents
  • Copyright
  • Dedication
  • Acknowledgments
  • Section I. Historical Perspective
  • 1. Brief History of the Cardiovascular System
  • Section II. Cardiovascular Development
  • 2. Embryology and Etiology
  • The First Week of Life
  • The Second Week of Life
  • The Third Week of Life
  • The Fourth Week of Life
  • The Fifth Week of Life
  • The Sixth and Seventh Weeks of Life
  • Abnormal Mammalian Cardiovascular Embryology: the Iv/Iv Mouse
  • Section III. Anatomic and Developmental Approach to Diagnosis
  • 3. Morphologic Anatomy
  • The Atria
  • 4. Segmental Anatomy
  • The Cardiac Segments
  • Atrial Situs
  • Ventricular Situs
  • Great Arterial Situs
  • Section IV. Congenital Heart Disease
  • 5. The Congenital Cardiac Pathology Database
  • 6. Systemic Venous Anomalies
  • Persistent Left or Right Superior Vena Cava
  • Interruption of the Inferior Vena Cava
  • Atresia or Stenosis of Coronary Sinus Ostium
  • Aneurysms of the Sinus Venosus
  • Absence or Atresia of the Right Superior Vena Cava
  • Absence of Left Innominate Vein
  • Innominate Vein Anterior to the Thymus
  • The Raghib Syndrome
  • Right Superior Vena Cava Draining Partly into the Left Atrium
  • Retroaortic Innominate Vein
  • Left-To-Right Switching of the Inferior Vena Cava
  • Umbilical Vein to Coronary Sinus
  • Anomalous “Portal” Vein to Azygos Vein
  • Inferior Vena Cava Never Connects Directly With Left Atrium
  • Summary
  • 7. Pulmonary Venous Anomalies
  • Totally Anomalous Pulmonary Venous Connection/Drainage
  • Obstruction
  • Classification of Totally Anomalous Pulmonary Venous Connection
  • Findings
  • Partially Anomalous Pulmonary Venous Connection/Drainage
  • Discussion of Partially Anomalous Pulmonary Venous Connection and Partially Anomalous Pulmonary Venous Drainage
  • Discussion and Literature Review on Partially Anomalous Pulmonary Venous Connection and Drainage
  • Pulmonary Arteriovenous Fistulae
  • Stenosis of the Pulmonary Veins
  • Pulmonary Veno-Occlusive Disease
  • Misalignment of Pulmonary Veins With Alveolar Capillary Dysplasia
  • Pulmonary Varix
  • Normal Anatomic Variations of the Pulmonary Veins
  • Recent Investigations Concerning the Development of the Pulmonary Veins
  • 8. Cor Triatriatum Sinistrum (Subdivided Left Atrium) and Cor Triatriatum Dextrum (Subdivided Right Atrium)
  • Definition
  • Cor Triatriatum Sinistrum
  • Embryology
  • Pathologic Anatomy
  • The Anomaly
  • Present Series
  • Literature and Discussion
  • Cor Triatriatum Dextrum
  • Present Series
  • Discussion and Literature
  • 9. Interatrial Communications
  • How Many Anatomic Types of Interatrial Communications Are There?
  • Ostium Secundum Type of Atrial Septal Defect
  • How Important Clinically Was Secundum Atrial Septal Defect II?
  • Device Closure of Secundum Atrial Septum Defects
  • Literature
  • Pathologic Anatomy
  • Diagnosis of Secundum Atrial Septal Defect
  • Surgery for Secundum Atrial Septal Defect
  • Transcatheter Intervention for Device Closure of Atrial Septal Defect Ii
  • Summary
  • 10. Juxtaposition of the Atrial Appendages
  • Definition
  • Material
  • Classification
  • Summary
  • 11. Common Atrioventricular Canal
  • Morphology and Classification
  • Embryology
  • Etiology
  • Parachute Mitral Valve
  • Double-Orifice Mitral Valve
  • Findings
  • Ventriculoarterial Alignments
  • Conus
  • The Ventricular Septal Defects
  • The Pulmonary Outflow Tract
  • Partial Forms of Common AV Canal
  • Segmental Anatomy
  • Comparison of Pulmonary Valvar Obstruction
  • Transitional Form of Common AV Canal
  • Ventricular Septal Defect of the AV Canal Type
  • Right Ventricle
  • Left Ventricle
  • Common AV Canal With Single Ventricle
  • Findings
  • Atrial Septum
  • Ventricular Septum
  • Segmental Anatomy
  • Systemic Veins
  • Pulmonary Veins
  • The Atria
  • Isolated Cleft of the Mitral Valve
  • Discussion
  • Summary
  • 12. Double-Outlet and Common-Outlet Right Atrium
  • Anatomic Types of Double-Outlet Right Atrium/Common-Outlet Right Atrium
  • Double-Outlet Right Atrium and Common-Outlet Right Atrium With Leftward Malalignment of the Atrial Septum
  • Other Cases of Dora
  • Summary
  • 13. Tricuspid Valve Anomalies
  • Ebstein’s Malformation
  • Study of Ebstein’s Anomaly of the Tricuspid Valve and Right Ventricle
  • Non-Ebstein Tricuspid Regurgitation
  • Summary
  • Tricuspid Atresia
  • Findings
  • Anatomic Details
  • Arrhythmias
  • Phylogeny
  • Congenital Tricuspid Stenosis
  • AV Discordance With VA Concordance
  • Discussion
  • Summary
  • Our Series
  • Patent Foramen Ovale
  • Pathology
  • Discussion
  • Multiple Fenestrations (“Filigree”) Tricuspid Valve
  • Literature
  • Chapter Summary
  • 14. Mitral Valve Anomalies
  • Mitral Atresia
  • Mitral Atresia With Normal Segmental Anatomy {S,D,S}
  • Mitral Atresia With Normal Segmental Anatomy {S,D,S}, Intact Ventricular Septum, and Aortic Valvular Atresia
  • Morphogenesis
  • Mitral Atresia {S,D,S}, Intact Ventricular Septum, and Patent Aortic Valve
  • Mitral Atresia {S,D,S}, Ventricular Septal Defect(s), and Patent Aortic Valve
  • Mitral Atresia {S,D,S}, Ventricular Septal Defect, and Aortic Valvar Atresia
  • Mitral Atresia {S,D,S}, Ventral Septal Defect, and Truncus Arteriosus
  • Mitral Atresia {S,D,S} With A Ventricular Septal Defect or A Bulboventricular Foramen, With A Large Left Ventricle and A Small Right Ventricle, or A Single Left Ventricle and an Absent Right Ventricle
  • Mitral Atresia {S,D,S}, Aortic Valvar Atresia, Tricuspid Atresia, and Pulmonary Valvar Aresia in A Conjoined Twin
  • Mitral Atresia With Double-Outlet Right Ventricle or Transposition of the Great Arteries in Visceroatrial Situs Solitus With Concordant D-Loop Ventricles
  • Mitral Atresia With Ventricular Septal Defect and Double-Outlet Right Ventricle {S,D,D/“S”}
  • Mitral Atresia With No Ventricular Septal Defect and Double-Oulet Right Ventricle {S,D,D}
  • Mitral Atresia With or Without A Ventricular Septal Defect and Transposition of the Great Arteries {S,D,D} (N = 5, Table 14.2, Type 10)
  • Mitral Atresia (Right-Sided) With Ventricual Septal Defect and Double-Outlet Right Ventricle {S, L, L} (n = 4, 2.26% of the Series)
  • Mitral Atresia (Right-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {S,L,L}
  • Anatomic Type 13 With Mitral Atresia (Right-Sided), Intact Ventricular Septum, and Double-Outlet Right Ventricle {I,L,L}
  • Mitral Atresia (Left-Sided) With or Without A Ventral Septal Defect and Double-Outlet Right Ventricle {I,D,D}
  • Mitral Atresia (Left-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {I,D,D/A}
  • Mitral Atresia With Double-Outlet Right Ventricle {A(S),D,D} and VISCERAL HETEROTAXY
  • Mitral Atresia With Double-Outlet Right Ventricle and D-Loop Ventricles in the Heterotaxy Syndrome With Asplenia
  • Mitral Atresia With Double-Outlet Right Ventricle and L-Loop Ventricles in the Heterotaxy Syndrome With Asplenia
  • Alignment Concordance and Discordance Versus Situs Concordance and Discordance
  • Occlusion of the Mitral Orifice By A Large, Ball-Like Rhabdomyoma Resulting in Stillbirth
  • Congenital Mitral Stenosis
  • Anatomic Types of Congenital Mitral Stenosis
  • Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome, That is, With Aortic Valvar Atresia or Severe Congenital Aortic Valvar Stenosis
  • Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome and Ventricular Septal Defect(s)
  • Multiple Congenital Anomalies
  • Anatomic Types of Parachute Mitral Valve
  • Revised Definition of Parachute Mitral Valve
  • The Conus (Infundibulum)
  • Trisomy
  • Congenital Mitral Stenosis With Ebstein’s Anomaly of the Tricuspid Valve
  • Dilated Cardiomyopathy
  • Forms of Marfan Syndrome
  • Congenital Mitral Regurgitation With Transposition of the Great Arteries
  • Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,L}
  • Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,A}
  • Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,L,L}
  • Congenital Mitral Regurgitation With Aberrant Left Coronary Artery from the Pulmonary Artery
  • Congenital Mitral Regurgitation With Hypertrophic Obstructive Cardiomyopathy
  • Congenital Mitral Regurgitation With Tetralogy of Fallot
  • Congenital Mitral Regurgitation With Single Left Ventricle
  • Congenital Mitral Regurgitation With Congenital Aneurysm or Dysplasia of the Left Ventricle
  • Hypertrophic and Hypoplastic Cardiomyopathy
  • Congenital Mitral Regurgitation With Down Syndrome
  • Congenital Mitral Regurgitation With Double-Outlet Right Ventricle
  • Congenital Mitral Regurgitation With Left Ventricular Outflow Tract Obstruction
  • Congenital Mitral Regurgitation With Duchenne Muscular Dystrophy
  • Congenital Mitral Regurgitation With Large Secundum Atrial Septal Defects
  • Congenital Mitral Regurgitation With Heterotaxy Syndrome and Polysplenia
  • Congenital Mitral Regurgitation With Hypoplastic Left Heart Syndrome
  • Congenital Absence of the Mitral Valve Leaflets, Also Known as Congenitally Unguarded Mitral Orifice
  • Adherent Mitral Valve Resulting in Fibrous Subaortic Stenosis
  • Mitral Dysplasia
  • Mitral Valve Hypoplasia Resulting Hemodynamically in Congenital Mitral Stenosis
  • Myxomatous Mitral Valve
  • Infantile Hypertrophic Cardiomyopathy
  • Congenital Mitral Valve Prolapse
  • Isolated Mitral Valve Prolapse
  • Nonisolated Mitral Valve Prolapse
  • Anatomic Considerations
  • 15. Infundibuloarterial Situs Equations: How Normally and Abnormally Related Great Arteries Are Built and the Importance of Infundibuloarterial Situs Concordance and Discordance
  • Solitus Normally Related Great Arteries1
  • Key to Symbolic Anatomy
  • Inverted Normally Related Great Arteries
  • Conal Situs
  • Discussion
  • Summary
  • Conclusions
  • 16. Ventricular Septal Defects
  • Components of the Ventricular Septum
  • Relative Frequencies of the Four Anatomic Types of Ventricular Septal Defect
  • Limitations of These Statistics
  • Ventricular Septal Defect With and Without Aortic Insufficiency
  • Summary
  • Ventral Septal Defects As Parts of A Conotruncal Malformation
  • 17. Single Ventricle
  • Single Left Ventricle With Infundibular Outlet Chamber
  • Single Right Ventricle
  • Rethinking Basic Definitions
  • Cardiac Geometry
  • Statistics
  • The Morphologic Anatomic Key to the Diagnostic Understanding of Single Ventricle
  • The Infundibular Apex and the Right Ventricular Sinus Apex
  • Diagnostic and Surgical Considerations
  • 18. Superoinferior Ventricles
  • Segmental Anatomy3-6
  • Status of the Atrioventricular Valves
  • Typical Superoinferior Ventricles With a Small Superior Right Ventricle
  • Developmental Hypothesis
  • Degrees of Right Ventricular Sinus Underdevelopment
  • Anatomic Types of Infundibulum
  • Superoinferior Ventricles With a Small Superior Left Ventricle and a Large Inferior Right Ventricle
  • The Morphogenesis of Superoinferior Ventricles
  • 19. Anomalous Infundibular Muscle Bundles: Also Known as Anomalous Muscle Bundles of the Right Ventricle and as Double-Chambered Right Ventricle
  • Statistics
  • Terminology
  • Findings
  • Discussion
  • Conclusions
  • 20. Tetralogy of Fallot
  • Early Pre-Fallot Reports
  • How Common is Tetralogy of Fallot?
  • What are the Situs of the Main Cardiac Segments in TOF?
  • Surgical Repair
  • Associated Malformations
  • Morphometric Study of TOF in the Neonatal Period18
  • Who was dr. Arthur Fallot and What did he Hope to Accomplish With his Publications in 1888?14,15
  • Fallot’s Conclusions15 (1888)
  • Why is the Tetralogy of Fallot Really the Monology of Stensen?
  • Infundibuloarterial Situs Equations and Tetralogy of Fallot20
  • Is TOF Related to Other Types of Congenital Heart Disease?
  • Explanation of Infundibuloarterial Situs Equations
  • Truncus Arteriosus28-30
  • Double-Outlet Left Ventricle31
  • 21. Absence of the Subpulmonary Infundibulum With Its Sequelae Has Been Misinterpreted as Common Aortopulmonary Trunk
  • Absence of the Infundibulum and its Sequelae
  • Other Findings
  • 22. Transposition of the Great Arteries
  • Transposition of the Great Arteries
  • Infundibuloarterial Situs Analysis
  • Chirality or Handedness
  • Errors: Bilaterally Right Atria and Bilaterally Left Atria
  • Coronary Arteries in TGA
  • Morphogenesis of TGA
  • Diagrams and Photographs
  • 23. Double-Outlet Right Ventricle
  • Definition and Differential Diagnosis
  • Findings
  • Discussion
  • Infundibulum in Group III Double-Outlet Right Ventricle
  • Classification of Double-Outlet Right Ventricle
  • The Taussig-Bing Malformation
  • Morphogenesis
  • The Infundibuloarterial Situs Equations
  • When the VSD is too Small
  • Double-Outlet Right Ventricle With Intact Ventricular Septum and Left Ventricle–to–Right Atrium Shunt
  • History
  • Summary of Double-Outlet Right Ventricle
  • Detailed Anatomic Types of Double-Outlet Right Ventricle (N = 73)
  • 24. Double-Outlet Left Ventricle
  • Why is Double-Oulet Left Ventricle so Rare?
  • What DOLV is not
  • Anatomic Types of Double-Outlet Left Ventricle
  • Discussion
  • Anatomic Types of Double-Outlet Left Ventricle
  • One Good Ventricle Heart
  • Segmental Anatomy
  • Infundibulum
  • Embryonic Morphogenetic Movements of the Great Arteries
  • Discussion
  • Mechanisms Resulting in Double-Outlet Left Ventricle
  • 25. Anatomically Corrected Malposition of the Great Arteries
  • What Causes Anatomically Corrected Malposition of the Great Arteries?
  • Anatomic Types of Anatomically Corrected Malposition of the Great Arteries
  • Surgery of Anatomically Corrected Malposition of the Great Arteries
  • Differential Diagnosis
  • Original Sources
  • Who First Discovered and Described Anatomically Corrected Malposition of the Great Arteries?
  • Summary
  • Infundibuloarterial Situs Equations
  • 26. What Prevents and What Permits the Embryonic Great Arterial Switch?
  • Basic Principles
  • Types of Situs
  • Grading Infundibular Development
  • Situs Equations
  • Double-Outlet Left Ventricle of the Paul Type19
  • Synergy
  • Evolution
  • Biophysics
  • Apoptosis
  • Conclusions
  • 27. Infundibuloarterial Situs Equations and Analysis
  • Infundibuloarterial Situs Concordance and Discordance
  • The Laws of the Great Arteries
  • Ventriculoarterial Alignment Concordance
  • Morphogenetic Movements of the Great Arteries
  • The Infundibulum
  • Infundibular Functions
  • Intermediate Cases
  • Laboratory Diagnosis of the Infundibulum
  • Evolution and Human Congenital Heart Disease
  • Right-Left Asymmetry
  • Embryonic Arterial Switches From Right Ventricle to Left Ventricle
  • The Probable Importance of Apoptosis
  • 28. The Cardiac Conduction System
  • The Sinoatrial Node
  • The Internodal and Interatrial Conduction Pathways
  • The Atrioventricular Node and the Atrioventricular Bundle
  • Historical Note
  • The Atrioventricular Node and Atrioventricular Bundle Continued
  • Surgical Relevance
  • The Communicating Atrioventricular Node
  • The Course of the Atrioventricular Bundle
  • The Atrioventricular Conduction System in Transposition of the Great Arteries {S,L,L}
  • Is the Cardiac Conduction System Invisible to the Naked Eye?
  • Literature Review
  • 29. The Heterotaxy Syndromes: Asplenia, Polysplenia, and With Normally Formed but Right-Sided Spleen
  • What are the Heterotaxy Syndromes?
  • What was the Atrial Situs?
  • What structures are Involved?
  • What is the Morphologically Right Atrium?
  • What is the Morphologically Left Atrium?
  • When can we not Diagnose the Atrial Situs?
  • What Does Double-Outlet Right Ventricle With Asplenia Look Like?
  • What Does the Heart in the Heterotaxy Syndrome With Polysplenia Look Like?
  • Relations Between the Great Arteries
  • Types of Atrial Situs
  • Literature
  • Management
  • Summary
  • 30. Conclusions
  • Sidedness of the Semilunar Valves
  • The Importance of Segmental Situs
  • Summary
  • Appendix 1. Understanding Normally and Abnormally Related Great Arteries
  • Post Scriptum
  • E-Appendix 2. Chronological List of Publications 1961 to 2021
  • Index