Congenital Heart Disease

A Clinical, Pathological, Embryological, and Segmental Analysis

1st Edition - April 6, 2022
Author: Richard Van Praagh
Language: English
Hardback ISBN:
9 7 8 - 1 - 5 6 0 5 3 - 3 6 8 - 9

Authored by the originator of the standard nomenclature for this spectrum of disorders, Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis… Read more

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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.

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

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