Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis

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CongenitalHeart DiseaseA Clinical, Pathological, Embryological,and Segmental Analysis
Congenital Heart Disease: A Clinical, Pathological, Embryological,and Segmental Analysis
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DEDICATION
ACKNOWLEDGMENTS
Contents
1 - Historical Perspective
1 - Brief History of the Cardiovascular System
REFERENCES
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
Embryology of the Aortic Arch System
ABNORMAL MAMMALIAN CARDIOVASCULAR EMBRYOLOGY: THE IV/IV MOUSE
Literature
Embryology of Specific Anomalies
Recent Etiologic Insights
REFERENCES
3 - Anatomic and Developmental Approach to Diagnosis
3 - Morphologic Anatomy
THE ATRIA
The Morphologically Right Atrium
The Morphologically Left Atrium
The Morphologically Right Ventricle
The Morphologically Left Ventricle
Variations of the Conus Arteriosus
Phylogenesis or Evolution of the Left and Right Ventricles
On the Importance of Examining Heart Specimens
REFERENCES
4 - Segmental Anatomy
THE CARDIAC SEGMENTS
ATRIAL SITUS
VENTRICULAR SITUS
GREAT ARTERIAL SITUS
A- Transposition and A- Malposition
Segmental Alignments
The Segmental Approach to Diagnosis
REFERENCES
IV - Congenital Heart Disease
5 - The Congenital Cardiac Pathology Database
REFERENCES
6 - Systemic Venous Anomalies
PERSISTENT LEFT OR RIGHT SUPERIOR VENA CAVA
Nonsyndromic Multiple Congenital Anomalies
Literature Review and Discussion
INTERRUPTION OF THE INFERIOR VENA CAVA
Literature Review and Discussion
ATRESIA OR STENOSIS OF CORONARY SINUS OSTIUM
Literature Review and Discussion
ANEURYSMS OF THE SINUS VENOSUS
Literature Review and Discussion
ABSENCE OR ATRESIA OF THE RIGHT SUPERIOR VENA CAVA
Literature Review
ABSENCE OF LEFT INNOMINATE VEIN
Conclusions
INNOMINATE VEIN ANTERIOR TO THE THYMUS
Conclusion
THE RAGHIB SYNDROME
Conclusion
RIGHT SUPERIOR VENA CAVA DRAINING PARTLY INTO THE LEFT ATRIUM
Discussion
RETROAORTIC INNOMINATE VEIN
Definitions
Summary
LEFT-TO-RIGHT SWITCHING OF THE INFERIOR VENA CAVA
Summary
UMBILICAL VEIN TO CORONARY SINUS
ANOMALOUS “PORTAL” VEIN TO AZYGOS VEIN
INFERIOR VENA CAVA NEVER CONNECTS DIRECTLY WITH LEFT ATRIUM
REFERENCES
7 - Pulmonary Venous Anomalies
TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION/DRAINAGE
Definition
Classification
Embryology
OBSTRUCTION
Nonisolated Totally Anomalous Pulmonary Venous Connection Without Heterotaxy
Nonisolated Totally Anomalous Pulmonary Venous Connection With Heterotaxy
CLASSIFICATION OF TOTALLY ANOMALOUS PULMONARY VENOUS CONNECTION
Surgical Considerations
FINDINGS
Anatomic Types of TAPVC/D
Associated Malformations
Systemic Veins
PARTIALLY ANOMALOUS PULMONARY VENOUS CONNECTION/DRAINAGE
Scimitar Syndrome
Diagnostic and Surgical Implications
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
Severe Pulmonary Vascular Obstructive Disease
Incomplete Scimitar Syndrome
Natural History of Totally Anomalous Pulmonary Venous Connection and Drainage
Mystery Cases
Left Atrial and Left Ventricular Volumes in Totally Anomalous Pulmonary Venous Connection and Drainage
Scimitar Syndrome Expanded?
Anomalous Pulmonary Venous Drainage With Pulmonary Atrioventricular Fistula(s)
Familial Totally Anomalous Pulmonary Venous Connection
Displacement of the “Atrial Septum” With Partially Anomalous Pulmonary Venous Connection and Drainage
Unilateral Emphysema With Totally Anomalous Pulmonary Venous Connection
Is Totally Anomalous Pulmonary Venous Connection Below the Diaphragm Always Obstructed?
Gastrointestinal Hemorrhage: Unusual Complication of Totally Anomalous Pulmonary Venous Connection
Surgical Correction of Totally Anomalous Pulmonary Venous Connection in Premature Neonates
Surgical Correction of Totally Anomalous Pulmonary Venous Connection Below the Diaphragm
Totally Anomalous Pulmonary Venous Connection: A Form of Cyanotic Congenital Heart Disease
Should Totally Anomalous Pulmonary Venous Connection Be Treated With Initial Balloon Atrial Septostomy?
The Essence of the Scimitar Syndrome
The Statistics of Pulmonary Venous Anomalies
Pulmonary Vascular Disease With Totally Anomalous Pulmonary Venous Connection
Rare Site of Obstruction in Totally Anomalous Pulmonary Venous Connection
Partially Anomalous Pulmonary Venous Connection and Drainage With Intact Atrial Septum: A Dangerous Duo
Prenatal Damage to Pulmonary Vascular Bed and to Extrapulmonary Veins in Obstructed Totally Anomalous Pulmonary Venous Connectio...
Does Left Ventricular Size Help Determine Survival in Totally Anomalous Pulmonary Venous Connection?
Surgical Experience With Totally Anomalous Pulmonary Venous Connection in Infancy
PULMONARY ARTERIOVENOUS FISTULAE
Cases
STENOSIS OF THE PULMONARY VEINS
Stenosis of Individual Pulmonary Veins
Stenosis of the Pulmonary Veins With Visceral Heterotaxy and Asplenia
Congenital Pulmonary Venous Stenosis With Totally Anomalous Pulmonary Venous Connection to the Left Innominate Vein
Congenital Pulmonary Venous Stenosis With Totally Anomalous Pulmonary Venous Connection Below the Diaphragm
Functional Pulmonary Venous Stenosis With the Scimitar Syndrome
Functional Stenosis of the Pulmonary Veins
Poor Incorporation of the Common Pulmonary Vein Into the Left Atrium
The Literature
PULMONARY VENO- OCCLUSIVE DISEASE
Atresia or Absence of the Pulmonary Veins
Pulmonary Vein Atresia
The Literature
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
REFERENCES
8 - Cor Triatriatum Sinistrum (Subdivided Left Atrium) and Cor Triatriatum Dextrum (Subdivided Right Atrium)
DEFINITION
COR TRIATRIATUM SINISTRUM
EMBRYOLOGY
PATHOLOGIC ANATOMY
THE ANOMALY
Pulmonary Vein Entrapment Hypothesis
Associated Malformations
PRESENT SERIES
LITERATURE AND DISCUSSION
COR TRIATRIATUM DEXTRUM
PRESENT SERIES
Prominent Right Venous Valve Versus Cor Triatriatum Dextrum
DISCUSSION AND LITERATURE
REFERENCES
9 - Interatrial Communications
HOW MANY ANATOMIC TYPES OF INTERATRIAL COMMUNICATIONS ARE THERE?
OSTIUM SECUNDUM TYPE OF ATRIAL SEPTAL DEFECT
Pentalogy of Fallot
Common Atrium
Aneurysm of Septum Primum
Embryology of Septum Primum
The Pathologic Anatomic Findings in 640 Human Cases of Secundum Atrial Septal Defect
HOW IMPORTANT CLINICALLY WAS SECUNDUM ATRIAL SEPTAL DEFECT II?
When Secundum Atrial Septal Defect II Was the Most Important Clinical Problem
When Atrial Septal Defect II Was an Important Part of the Patient’s Main Clinical Problem
When Secundum Atrial Septal Defect II Is Present, but Not the Patient’s Primary Clinical Problem
Raghib Syndrome Without a Large Low Posterior Interatrial Communication
The Pathologic Anatomy of Secundum Atrial Septal Defects
DEVICE CLOSURE OF SECUNDUM ATRIAL SEPTUM DEFECTS
Summary of the Anatomic Types of Secundum Atrial Septal Defect
LITERATURE
Who First Described the Ostium Secundum Type of Atrial Septal Defect?
PATHOLOGIC ANATOMY
Hemodynamic Changes of the Atrioventricular Valves
Measurements of the Atrial Septal Defect II
Conduction System Abnormalities
Natural History of Secundum Atrial Septal Defect
Familial Secundum Atrial Septal Defect
Patent Foramen Ovale
Right- to- Left Shunt With a Secundum Atrial Septal Defect, Without Right- Sided Hypertension
Syndromes With Secundum Atrial Septal Defect
DIAGNOSIS OF SECUNDUM ATRIAL SEPTAL DEFECT
SURGERY FOR SECUNDUM ATRIAL SEPTAL DEFECT
TRANSCATHETER INTERVENTION FOR DEVICE CLOSURE OF ATRIAL SEPTAL DEFECT II
Cryptogenic Stroke and Migraine
Migraine
SUMMARY
REFERENCES
10 - Juxtaposition of the Atrial Appendages
DEFINITION
MATERIAL
CLASSIFICATION
Positional Classification
Anatomic Types of Juxtaposition of the Atrial Appendages
The Syndrome of JAA With Malposition of the RAA39
Summary of JRAA
Discussion of JRAA
Atrioventricular Malalignment in JAA
Morphogenesis of JAA
Morphogenetic Summary
Clinical and Surgical Relevance of Juxtaposition of the Atrial Appendages
Conclusions
REFERENCES
11 - Common Atrioventricular Canal
MORPHOLOGY AND CLASSIFICATION
Type A Completely Common AV Canal
Type B Completely Common AV Canal
Type C Completely Common AV Canal
Partial Forms of Common AV Canal
EMBRYOLOGY
ETIOLOGY
PARACHUTE MITRAL VALVE
DOUBLE- ORIFICE MITRAL VALVE
Double- Orifice Mitral Valve Principles
FINDINGS
Complete Forms of Common AV Canal
Associated Anomalies
Heart Position
Types of Ventricular Loop
Types of AV Alignment
VENTRICULOARTERIAL ALIGNMENTS
Conclusions
CONUS
Segmental Sets or Combinations
The Common AV Valve and the AV Septal Defect
Additional Interatrial Communications
THE VENTRICULAR SEPTAL DEFECTS
The Right Ventricle
The Left Ventricle
THE PULMONARY OUTFLOW TRACT
The Aortic Outflow Tract
The Pulmonary Valve
The Aortic Valve
Pulmonary Arteries
Coronary Arteries
Aortic Arch
Conclusions
PARTIAL FORMS OF COMMON AV CANAL
Heart Position
Conclusions
Gender
Age at Death
Comparison of the Partial and Complete Forms of Common AV Canal
SEGMENTAL ANATOMY
The Pulmonary Outflow Tract in Partially Common AV Canal
COMPARISON OF PULMONARY VALVAR OBSTRUCTION
The Aortic Outflow Tract in Partially Common AV Canal
Aortic Valve
Pulmonary Arteries
Coronary Arteries
Aortic Arch
Aortic Isthmus
TRANSITIONAL FORM OF COMMON AV CANAL
VENTRICULAR SEPTAL DEFECT OF THE AV CANAL TYPE
RIGHT VENTRICLE
LEFT VENTRICLE
Pulmonary Outflow Tract
Aortic Outflow Tract
Pulmonary Valve
Aortic Valve
Coronary Arteries
Aortic Arch
Aortic Isthmus
Brachiocephalic Arteries
Ductus Arteriosus
Associated Anomalies
COMMON AV CANAL WITH SINGLE VENTRICLE
FINDINGS
Types of Single Ventricle
ATRIAL SEPTUM
VENTRICULAR SEPTUM
The AV Valve(s)
SEGMENTAL ANATOMY
Conus
SYSTEMIC VEINS
PULMONARY VEINS
Lobation of Lungs
THE ATRIA
Pulmonary Outflow Tract
Pulmonary Valve
Aortic Outflow Tract
Aortic Valve
Pulmonary Artery
Coronary Arteries
Aortic Arch
Aortic Isthmus
Brachiocephalic Arteries
Ductus Arteriosus
ISOLATED CLEFT OF THE MITRAL VALVE
Summary of Findings
DISCUSSION
Are Our Data Accurately Representative?
Terminology
REFERENCES
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
Double-Outlet Right Atrium With Rightward Malalignment of the Ventricular Septum
Double-Outlet Right Atrium With an Accessory Valve Between the Coronary Sinus and the Left Ventricle
OTHER CASES OF DORA
REFERENCES
13 - Tricuspid Valve Anomalies
EBSTEIN’S MALFORMATION
STUDY OF EBSTEIN’S ANOMALY OF THE TRICUSPID VALVE AND RIGHT VENTRICLE
Ebstein’s Anomaly With Tricuspid Regurgitation
Ebstein’s Anomaly With Congenital Tricuspid Stenosis
Ebstein’s Anomaly With Tricuspid Atresia (Imperforate Ebstein’s)
The Problem of Borderline Cases
With Tricuspid Regurgitation
Left- Sided Ebstein’s Anomaly
Findings
Tricuspid Valve Function in Ebstein’s Anomaly
Types of Literature
NON- EBSTEIN TRICUSPID REGURGITATION
Tricuspid Regurgitation With Pulmonary Atresia and Intact Ventricular Septum
Non- Ebstein Tricuspid Regurgitation With Double- Inlet Left Ventricle
State of the Right Ventricular Sinus
Non- Ebstein Regurgitation Associated With Hypoplastic Left Heart Syndrome
Congenital Non- Ebstein Tricuspid Regurgitation Associated With Trisomies
Non- Ebstein Congenital Tricuspid Regurgitation in Marfan Syndrome
Congenital Non- Ebstein Tricuspid Regurgitation With Uhl’s Disease
Congenital Polyvalvar Disease With Congenital Non- Ebstein Tricuspid Regurgitation
Congenital Tricuspid Regurgitation Associated With Transposition of the Great Arteries {S,D,D} and (Usually) Dysplastic Tricuspi...
Tricuspid Valve Prolapse Causing Congenital Non- Ebstein Tricuspid Regurgitation
Dysplastic Right Ventricle and Tricuspid Valve
Double- Inlet and Double- Outlet Right Ventricle (DIDO RV) With Congenital Non- Ebstein Tricuspid Regurgitation
Congenitally Unguarded Tricuspid Orifice
Downward Displacement of the Anterior Tricuspid Leaflet With Blood Cysts of the Tricuspid Valve Leaflets
Congenital Mitral Regurgitation With Pulmonary Hypertension, Pulmonary Vascular Obstructive Disease, and Tricuspid Regurgitation...
Hypoplastic Pulmonary Arteries With Pulmonary Artery Hypertension and Congenital Tricuspid Regurgitation
SUMMARY
TRICUSPID ATRESIA
Classification
FINDINGS
Classifications
ANATOMIC DETAILS
Tricuspid Atresia {S,D,S} with Pulmonary Outflow Tract Stenosis
Tricuspid Atresia With D- Loop Transposition of the Great Arteries
Pulmonary Outflow Tract Obstruction
Tricuspid Atresia {S,D,S} Without Pulmonary Outflow Tract Obstruction
Tricuspid Atresia {S,D,S} With Pulmonary Outflow Tract Atresia
Tricuspid Atresia With Truncus Arteriosus
Tricuspid Atresia With Double- Outlet Right Ventricle {S,D,D}
Findings
Tricuspid Atresia in Conjoined Twins
Discussion of Typical Tricuspid Atresia
ARRHYTHMIAS
Surgical Implications
PHYLOGENY
What Is Tricuspid Atresia From the Phylogenetic and Ontogenetic Viewpoints?
What Is the Significance of Right Ventricular Evolution to Human Congenital Heart Disease?
CONGENITAL TRICUSPID STENOSIS
Small- Volume Right Ventricular Sinus Cavity
Other Organ Systems Involved
Transposition of the Great Arteries
Congenital Tricuspid Stenosis Without Severe Pulmonary Stenosis
Congenital Tricuspid Stenosis With Tetralogy of Fallot
AV DISCORDANCE WITH VA CONCORDANCE
Complete Form of Common AV Canal With Congenital Tricuspid Stenosis
Double- Outlet Right Ventricle
Congenital Tricuspid Stenosis With Ebstein’s Anomaly
Ebstein’s Anomaly: Additional Thoughts
DISCUSSION
Mechanisms Resulting in Congenital Tricuspid Stenosis
SUMMARY
Congenitally Unguarded Tricuspid Orifice, Also Known as Congenital Absence of Tricuspid Leaflets and Tensor Apparatus
OUR SERIES
PATENT FORAMEN OVALE
Status Post–Waterston Anastomosis
Hypoplastic Tricuspid Valve
Myxomatous Tricuspid Valve
Tricuspid Valve Prolapse
PATHOLOGY
Muscular Tricuspid Valve
Double- Orifice Tricuspid Valve
Valvar Aortic Atresia With Intact Ventricular Septum and Double- Orifice Tricuspid Valve
Double- Inlet Left Ventricle With Double- Orifice Tricuspid Valve
Pathogenic Mechanism of DOTV
DISCUSSION
Triple- Orifice Tricuspid Valve
MULTIPLE FENESTRATIONS (“FILIGREE”) TRICUSPID VALVE
LITERATURE
Straddling Tricuspid Valve
Attachments of the Straddling Tricuspid Valve219
Other Topics
Can Pectus Excavatum Cause Tricuspid Compression?
CHAPTER SUMMARY
REFERENCES
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
Salient Anatomic Features
Why Did This Anatomic Type of Mitral Atresia Have Such an Unfavorable History With a Median Age at Death of Only 10 Days
Morphogenesis
Mitral Atresia {S,D,S}, Intact Ventricular Septum, and Patent Aortic Valve
Anatomic Findings
Mitral Atresia {S,D,S}, Ventricular Septal Defect(s), and Patent Aortic Valve
Salient Anatomic Features
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 R...
Salient Anatomic Features
Ventricular Anatomy
Developmental Hypothesis
Summary
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 Co...
Mitral Atresia With Ventricular Septal Defect and Double- Outlet Right Ventricle {S,D,D/“S”}
Anatomic Features
Mitral Atresia With No Ventricular Septal Defect and Double- Oulet Right Ventricle {S,D,D}
Anatomic Features
Mitral Atresia With or Without A Ventricular Septal Defect and Transposition of the Great Arteries {S,D,D} (N = 5, Table 14.2, T...
Anatomic Features
Mitral Atresia (Right- Sided) With Ventricual Septal Defect and Double-ŁOutlet Right Ventricle {S, L, L} (n = 4, 2.26% of the Se...
Anatomic Features
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-ŁOutlet Right Ventricle {I,L,L}
Mitral Atresia (Left- Sided) With or Without A Ventral Septal Defect and Double- Outlet Right Ventricle {I,D,D}
Anatomic Features
Mitral Atresia (Left- Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {I,D,D/A}
Anatomic Features
Mitral Atresia With Double- Outlet Right Ventricle {A(S),D,D} and VISCERAL HETEROTAXY
Heterotaxy With Polysplenia
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
Summary and Discussion: Pathologic Anatomy of Mitral Atresia
What the Literature Teaches Concerning the Pathologic Anatomy of Mitral Atresia
Classification
AV and VA “Connections”
Alignment Concordance and Discordance Versus Situs Concordance and Discordance
Occlusion of the Mitral Orifice By A Large, Ball- Like Rhabdomyoma Resulting in Stillbirth
Usage
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...
Aortic Valvar Atresia Versus Aortic Valvar Stenosis
Other Features of Interest
Other Categories Presented in Table 14.13
Left Ventricular Myocardial Dysplasia With Diverticulum or Diverticula
Prominent Anterolateral Muscle of the Left Ventricle (Muscle of Moulaert)
Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome and Ventricular Septal Defect(s)
What Is Congenital Mitral Stenosis
Shone Syndrome Variants
Summary
Congenital Mitral Stenosis Without the Hypoplastic Left Heart Syndrome and Without Other Major Atrioventricular Anomalies
Multiple Congenital Anomalies
Phenotypes
Trisomies
Syndromes
Other Findings
Typical Congenital Mitral Stenosis Without the Hypoplastic Left Heart Syndrome
The Morphologic Features of “Typical” Congenital Mitral Stenosis (Without Hypoplastic Left Heart Syndrome)
Parachute Mitral Valve in Patients With Congenital Mitral Stenosis and Without Hypoplastic Left Heart Syndrome
Anatomic Types of Parachute Mitral Valve
Single Anterolateral Papillary Muscle Receiving All Chordae Tendineae
Revised Definition of Parachute Mitral Valve
Single Posteromedial Papillary Muscle Receiving All Chordae Tendineae
Single Papillary Muscle Resulting From Fusion of Both Papillary Muscle Groups With a Single Focus of Chordal Insertion
Parachute Mitral Valve with Two Partially Fused Papillary Muscle Groups
Congenital Mitral Stenosis With Double- Outlet Right Ventricle
The Conus (Infundibulum)
Left Ventricular Outflow Tract Obstruction in Patients With Congenital Mitral Stenosis but Without the Hypoplastic Left Heart Sy...
Trisomy
Double- Orifice Mitral Valve
Congenital Mitral Stenosis With Ebstein’s Anomaly of the Tricuspid Valve
Congenital Mitral Regurgitation
Material and Methods
Causes of Congenital Mitral Regurgitation
Incomplete Form of Common Atrioventricular Canal With Cleft Mitral Valve
Severe Aortic Valvar Stenosis or Aortic Valvar Atresia
Dilated Cardiomyopathy
Marfan Syndrome With or Without Other Congenital Anomalies
Summary
Forms of Marfan Syndrome
Congenital Mitral Regurgitation With Transposition of the Great Arteries
Summary
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}
Summary
Congenital Mitral Regurgitation With Aberrant Left Coronary Artery from the Pulmonary Artery
Features Found With Aberrant Left Coronary Artery Arising From the Pulmonary Artery
Congenital Mitral Regurgitation With Hypertrophic Obstructive Cardiomyopathy
Clinical Profile
Congenital Mitral Regurgitation With Tetralogy of Fallot
Clinical and Anatomic Features
Summary
Congenital Mitral Regurgitation With Single Left Ventricle
Congenital Mitral Regurgitation With Congenital Aneurysm or Dysplasia of the Left Ventricle
Hypertrophic and Hypoplastic Cardiomyopathy
Histologic Findings of Congenital Left Ventricular Aneurysm
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 Straddling Mitral Valve
Congenital Mitral Regurgitation With Truncus Arteriosus
Mitral Regurgitation With Pulmonary Arterial Vascular Obstructive Disease and Pulmonary Veno- occlusive Disease
Congenital Mitral Regurgitation With Heterotaxy Syndrome and Polysplenia
Congenital Mitral Regurgitation With Hypoplastic Left Heart Syndrome
Mitral Regurgitation With a High, Subaortic Ventricular Septal Defect
Social Comment
Other Mitral Valve Anomalies
Congenital Absence of the Mitral Valve Leaflets, Also Known as Congenitally Unguarded Mitral Orifice
Adherent Mitral Valve Resulting in Fibrous Subaortic Stenosis
Comment
Mitral Dysplasia
Comment
Mitral Valve Hypoplasia Resulting Hemodynamically in Congenital Mitral Stenosis
Mitral Valve Anomaly Causing Left Ventricular Outflow Tract Obstruction: Fibrous Left Ventricular Outflow Tract Stenosis or Atre...
Myxomatous Mitral Valve
Salient Autopsy Findings
Comment
Infantile Hypertrophic Cardiomyopathy
Comment
Comment
Comment
Congenital Mitral Valve Prolapse
Isolated Mitral Valve Prolapse
Nonisolated Mitral Valve Prolapse
Quantitation
Anatomic Considerations
Mitral Valve Replacement in Infants
Summary
References
15 - Infundibuloarterial Situs Equations: How Normally and Abnormally Related Great Arteries Are Built and the Importance of Infundib...
Solitus Normally Related Great Arteries1
Key to Symbolic Anatomy
Inverted Normally Related Great Arteries
Transposition of the Great Arteries
Double- Outlet Right Ventricle
Double- Outlet Left Ventricle
Conal Situs
Tetralogy of Fallot
Truncus Arteriosus
Discussion
Historical Note
Summary
Conclusions
References
16 - VENTRICULAR SEPTAL DEFECTS
COMPONENTS OF THE VENTRICULAR SEPTUM
Anatomic Types of Ventricular Septal Defect
RELATIVE FREQUENCIES OF THE FOUR ANATOMIC TYPES OF VENTRICULAR SEPTAL DEFECT
LIMITATIONS OF THESE STATISTICS
VENTRICULAR SEPTAL DEFECT WITH AND WITHOUT AORTIC INSUFFICIENCY
The Essential Facts
SUMMARY
Apical Ventricular Septal Defects
The Story of Ventricular Septal Defects
Dr. Galey’s Conclusions104
Groups Ia and Ib: Normal Pulmonary Artery Pressure
VENTRAL SEPTAL DEFECTS AS PARTS OF A CONOTRUNCAL MALFORMATION
REFERENCES
17 - Single Ventricle
SINGLE LEFT VENTRICLE WITH INFUNDIBULAR OUTLET CHAMBER
SINGLE RIGHT VENTRICLE
RETHINKING BASIC DEFINITIONS
Single Right Ventricle With Double- Outlet Right Ventricle {I,L,I}
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
REFERENCES
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 Ventricular Septal Defect1
ANATOMIC TYPES OF INFUNDIBULUM
SUPEROINFERIOR VENTRICLES WITH A SMALL SUPERIOR LEFT VENTRICLE AND A LARGE INFERIOR RIGHT VENTRICLE
THE MORPHOGENESIS OF SUPEROINFERIOR VENTRICLES
REFERENCES
19 - Anomalous Infundibular Muscle Bundles: Also Known as Anomalous Muscle Bundles of the Right Ventricle and as Double- Chambered Right Ventricle
Anomalous Infundibular Muscle Bundles
STATISTICS
TERMINOLOGY
FINDINGS
DISCUSSION
CONCLUSIONS
REFERENCES
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
REFERENCES
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
Subpulmonary Infundibular Absence
REFERENCES
22 - Transposition of the Great Arteries
INFUNDIBULOARTERIAL SITUS ANALYSIS
Terminology
History
CHIRALITY OR HANDEDNESS
ERRORS: BILATERALLY RIGHT ATRIA AND BILATERALLY LEFT ATRIA
Alignments and Connections
Pathologic Anatomy of TGA
CORONARY ARTERIES IN TGA
MORPHOGENESIS OF TGA
DIAGRAMS AND PHOTOGRAPHS
TGA With Posterior Aorta, Anterior Pulmonary Artery, Subpulmonary Infundibulum, and Aortic- Atrioventricular Fibrous Continuity2...
REFERENCES
23 - Double- Outlet Right Ventricle
DEFINITION AND DIFFERENTIAL DIAGNOSIS
FINDINGS
DISCUSSION
Groups I, II, and III: A Segmental Classification
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
Double- Outlet Right Ventricle {S,D,L} With Subaortic Ventricular Septal Defect, Bilateral Infundibulum, and Pulmonary Stenosis
Double- Outlet Right Ventricle {S,D,L} With Pulmonary Stenosis
HISTORY
SUMMARY OF DOUBLE- OUTLET RIGHT VENTRICLE
Groups
Anatomic Types
DETAILED ANATOMIC TYPES OF DOUBLE- OUTLET RIGHT VENTRICLE (N = 73)
REFERENCES
24 - Double- Outlet Left Ventricle
WHY IS DOUBLE- OULET LEFT VENTRICLE SO RARE?
Definition
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
REFERENCES
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
REFERENCES
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
REFERENCES
27 - INFUNDIBULOARTERIAL SITUS EQUATIONS AND ANALYSIS
THE LAWS OF THE GREAT ARTERIES
VENTRICULOARTERIAL ALIGNMENT CONCORDANCE
MORPHOGENETIC MOVEMENTS OF THE GREAT ARTERIES
THE INFUNDIBULUM
INFUNDIBULAR FUNCTIONS
Infundibular Anatomic Opposites
Infundibular Anatomic Sameness
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
REFERENCES
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
What Did the Authors Conclude?
REFERENCES
29 - THE HETEROTAXY SYNDROMES: ASPLENIA, POLYSPLENIA, AND WITH NORMALLY FORMED BUT RIGHT- SIDED SPLEEN
WHAT WAS THE ATRIAL SITUS?
The Essence of the Heterotaxy Syndromes
WHAT STRUCTURES ARE INVOLVED?
How Many Kinds of Atrial Situs are There?
WHAT IS THE MORPHOLOGICALLY RIGHT ATRIUM?
WHAT IS THE MORPHOLOGICALLY LEFT ATRIUM?
WHEN CAN WE NOT DIAGNOSE THE ATRIAL SITUS?
Septum Primum Can Be Malpositioned
Findings of Heterotaxy Syndrome With Asplenia
Lobation of the Lungs
Types of Relationship Between the Great Arteries
Double- Outlet Right Ventricle With Asplenia
WHAT DOES DOUBLE- OUTLET RIGHT VENTRICLE WITH ASPLENIA LOOK LIKE?
Heterotaxy Syndrome With Asplenia and Transposition of the Great Arteries
Ventriculoarterial Concordance With Asplenia
The Superior Vena(e) Cava(e) With Asplenia
Inferior Vena Cava With Asplenia
Pulmonary Veins in Asplenia Syndrome
Patients With Aortic Outflow Tract Atresia
WHAT DOES THE HEART IN THE HETEROTAXY SYNDROME WITH POLYSPLENIA LOOK LIKE?
Visceral Heterotaxy With Normally Formed Spleen
Comparison of Asplenia and Polysplenia
RELATIONS BETWEEN THE GREAT ARTERIES
The Superior Vena Cava
The Inferior Vena Cava
The Atrial Appendages
TYPES OF ATRIAL SITUS
Atrioventricular Alignments
Ventricular Loop
Aortic Arch
Ductus Arteriosus
The Atrioventricular Valves
Midline- Associated Extracardiac Anomalies
LITERATURE
Diagnosis of the Heterotaxy Syndromes
MANAGEMENT
SUMMARY
REFERENCES
30 - Conclusions
SIDEDNESS OF THE SEMILUNAR VALVES
THE IMPORTANCE OF SEGMENTAL SITUS
Summary
REFERENCES
1 - Understanding Normally and Abnormally Related Great Arteries
GREAT ARTERIAL EQUATIONS*
Normally Related Great Arteries With or Without Associated Malformations
Tetralogy of Fallot
Situs of inf + GA
Inverted or L-Loop Hearts
CORRECT
Understanding of Truncus Arteriosus Communis
ABNORMALLY RELATED GREAT ARTERIES
D-Transposition of the Great Arteries
L-Transposition of the Great Arteries
Double-Outlet Right Ventricle
Double-Outlet Left Ventricle, Paul Type
Isolated Ventricular Inversion
BASIC PRINCIPLES (ADDRESSED THUS FAR)
THE EMBRYONIC ARTERIAL SWITCH
How Does It Happen Normally?
How Does It Happen in D-TGA?
How Does It Happen in DORV?
Central Role of the Subarterial Infundibulum
Rotation
Rotations, D- and L-
Equation 1
Equation 2 (inversus heart)
Synergy
SUMMARY
ACKNOWLEDGMENTS
REFERENCES
2 - POST SCRIPTUM
CONGENITAL HEART DISEASES AS FAILURES OF EVOLUTIONARY ADAPTATIONS
TETRALOGY OF FALLOT
CONGENITAL HEART DISEASES ASSOCIATED WITH EVOLUTIONARY FAILURE
E-2 - CHRONOLOGICAL LIST OF PUBLICATIONS 1961 TO 2021
1962
1963
1964
1965
1966
1967
1968
1969
1970
1971
1972
1973
1974
1975
1976
1977
1978
1979
1980
1981
1982
1983
1984
1985
1986
1987
1988
1989
1990
1991
1992
1993
1994
1995
1996
1997
1998
1999
2000
2001
2002
2003
2004
2005
2006
2007
2009
2010
2011
2012
2013
2015
2016
2017
2019
2021
INDEX
A
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
Y
Z
ibc