Congenital Heart Disease, E-Book , livre ebook

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

Richard Van Praagh, MD
Professor of Pathology Emeritus, Harvard Medical School, Director of the Cardiac Registry Lab Emeritus, Research Associate in Cardiac Surgery Emeritus, Emeritus Member of the Departments of Cardiology, Pathology, and Cardiac Surgery, Boston Children’s Hospital, Boston, Massachusetts
Table of Contents
Cover image
Title page
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. Ventr