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.
1. Cover image - p. i 2. Title page - p. ii 3. Table of Contents - p. iii 4. Copyright - p. iv 5. Dedication - p. v 6. Acknowledgments - p. vii 7. Section I. Historical Perspective - p. vii 8. 1. Brief History of the Cardiovascular System - p. 2 9. Section II. Cardiovascular Development - p. 11 10. 2. Embryology and Etiology - p. 14 11. Section III. Anatomic and Developmental Approach to Diagnosis - p. 58 12. 3. Morphologic Anatomy - p. 60 13. 4. Segmental Anatomy - p. 89 14. Section IV. Congenital Heart Disease - p. 104 15. 5. The Congenital Cardiac Pathology Database - p. 106 16. 6. Systemic Venous Anomalies - p. 113 17. Persistent Left or Right Superior Vena Cava - p. 113 18. Interruption of the Inferior Vena Cava - p. 123 19. Atresia or Stenosis of Coronary Sinus Ostium - p. 126 20. Aneurysms of the Sinus Venosus - p. 130 21. Absence or Atresia of the Right Superior Vena Cava - p. 133 22. Absence of Left Innominate Vein - p. 135 23. Innominate Vein Anterior to the Thymus - p. 136 24. The Raghib Syndrome - p. 137 25. Right Superior Vena Cava Draining Partly into the Left Atrium - p. 138 26. Retroaortic Innominate Vein - p. 140 27. Left-To-Right Switching of the Inferior Vena Cava - p. 142 28. Umbilical Vein to Coronary Sinus - p. 142 29. Anomalous “Portal” Vein to Azygos Vein - p. 142 30. Inferior Vena Cava Never Connects Directly With Left Atrium - p. 144 31. Summary - p. 151 32. 7. Pulmonary Venous Anomalies - p. 155 33. Totally Anomalous Pulmonary Venous Connection/Drainage - p. 155 34. Obstruction - p. 156 35. Classification of Totally Anomalous Pulmonary Venous Connection - p. 166 36. Findings - p. 172 37. Partially Anomalous Pulmonary Venous Connection/Drainage - p. 177 38. Discussion of Partially Anomalous Pulmonary Venous Connection and Partially Anomalous Pulmonary Venous Drainage - p. 192 39. Discussion and Literature Review on Partially Anomalous Pulmonary Venous Connection and Drainage - p. 194 40. Pulmonary Arteriovenous Fistulae - p. 206 41. Stenosis of the Pulmonary Veins - p. 209 42. Pulmonary Veno-Occlusive Disease - p. 216 43. Misalignment of Pulmonary Veins With Alveolar Capillary Dysplasia - p. 222 44. Pulmonary Varix - p. 222 45. Normal Anatomic Variations of the Pulmonary Veins - p. 222 46. Recent Investigations Concerning the Development of the Pulmonary Veins - p. 223 47. 8. Cor Triatriatum Sinistrum (Subdivided Left Atrium) and Cor Triatriatum Dextrum (Subdivided Right Atrium) - p. 234 48. 9. Interatrial Communications - p. 266 49. How Many Anatomic Types of Interatrial Communications Are There? - p. 266 50. Ostium Secundum Type of Atrial Septal Defect - p. 266 51. How Important Clinically Was Secundum Atrial Septal Defect II? - p. 278 52. Device Closure of Secundum Atrial Septum Defects - p. 298 53. Literature - p. 300 54. Pathologic Anatomy - p. 302 55. Diagnosis of Secundum Atrial Septal Defect - p. 306 56. Surgery for Secundum Atrial Septal Defect - p. 307 57. Transcatheter Intervention for Device Closure of Atrial Septal Defect Ii - p. 307 58. Summary - p. 309 59. 10. Juxtaposition of the Atrial Appendages - p. 320 60. 11. Common Atrioventricular Canal - p. 352 61. Morphology and Classification - p. 353 62. Embryology - p. 366 63. Etiology - p. 369 64. Parachute Mitral Valve - p. 370 65. Double-Orifice Mitral Valve - p. 374 66. Findings - p. 376 67. Ventriculoarterial Alignments - p. 377 68. Conus - p. 378 69. The Ventricular Septal Defects - p. 383 70. The Pulmonary Outflow Tract - p. 384 71. Partial Forms of Common AV Canal - p. 386 72. Segmental Anatomy - p. 387 73. Comparison of Pulmonary Valvar Obstruction - p. 390 74. Transitional Form of Common AV Canal - p. 391 75. Ventricular Septal Defect of the AV Canal Type - p. 391 76. Right Ventricle - p. 392 77. Left Ventricle - p. 393 78. Common AV Canal With Single Ventricle - p. 394 79. Findings - p. 395 80. Atrial Septum - p. 395 81. Ventricular Septum - p. 395 82. Segmental Anatomy - p. 397 83. Systemic Veins - p. 398 84. Pulmonary Veins - p. 398 85. The Atria - p. 399 86. Isolated Cleft of the Mitral Valve - p. 401 87. Discussion - p. 401 88. Summary - p. 406 89. 12. Double-Outlet and Common-Outlet Right Atrium - p. 416 90. 13. Tricuspid Valve Anomalies - p. 429 91. Ebstein’s Malformation - p. 429 92. Study of Ebstein’s Anomaly of the Tricuspid Valve and Right Ventricle - p. 435 93. Non-Ebstein Tricuspid Regurgitation - p. 462 94. Summary - p. 484 95. Tricuspid Atresia - p. 485 96. Findings - p. 488 97. Anatomic Details - p. 490 98. Arrhythmias - p. 518 99. Phylogeny - p. 519 100. Congenital Tricuspid Stenosis - p. 524 101. AV Discordance With VA Concordance - p. 533 102. Discussion - p. 543 103. Summary - p. 546 104. Our Series - p. 548 105. Patent Foramen Ovale - p. 548 106. Pathology - p. 553 107. Discussion - p. 563 108. Multiple Fenestrations (“Filigree”) Tricuspid Valve - p. 565 109. Literature - p. 566 110. Chapter Summary - p. 575 111. 14. Mitral Valve Anomalies - p. 594 112. Mitral Atresia - p. 594 113. Mitral Atresia With Normal Segmental Anatomy {S,D,S} - p. 597 114. Mitral Atresia With Normal Segmental Anatomy {S,D,S}, Intact Ventricular Septum, and Aortic Valvular Atresia - p. 597 115. Morphogenesis - p. 606 116. Mitral Atresia {S,D,S}, Intact Ventricular Septum, and Patent Aortic Valve - p. 608 117. Mitral Atresia {S,D,S}, Ventricular Septal Defect(s), and Patent Aortic Valve - p. 609 118. Mitral Atresia {S,D,S}, Ventricular Septal Defect, and Aortic Valvar Atresia - p. 614 119. Mitral Atresia {S,D,S}, Ventral Septal Defect, and Truncus Arteriosus - p. 615 120. 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 - p. 615 121. Mitral Atresia {S,D,S}, Aortic Valvar Atresia, Tricuspid Atresia, and Pulmonary Valvar Aresia in A Conjoined Twin - p. 628 122. Mitral Atresia With Double-Outlet Right Ventricle or Transposition of the Great Arteries in Visceroatrial Situs Solitus With Concordant D-Loop Ventricles - p. 632 123. Mitral Atresia With Ventricular Septal Defect and Double-Outlet Right Ventricle {S,D,D/“S”} - p. 632 124. Mitral Atresia With No Ventricular Septal Defect and Double-Oulet Right Ventricle {S,D,D} - p. 636 125. 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) - p. 640 126. Mitral Atresia (Right-Sided) With Ventricual Septal Defect and Double-Outlet Right Ventricle {S, L, L} (n = 4, 2.26% of the Series) - p. 641 127. Mitral Atresia (Right-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {S,L,L} - p. 643 128. Anatomic Type 13 With Mitral Atresia (Right-Sided), Intact Ventricular Septum, and Double-Outlet Right Ventricle {I,L,L} - p. 643 129. Mitral Atresia (Left-Sided) With or Without A Ventral Septal Defect and Double-Outlet Right Ventricle {I,D,D} - p. 645 130. Mitral Atresia (Left-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {I,D,D/A} - p. 646 131. Mitral Atresia With Double-Outlet Right Ventricle {A(S),D,D} and VISCERAL HETEROTAXY - p. 647 132. Mitral Atresia With Double-Outlet Right Ventricle and D-Loop Ventricles in the Heterotaxy Syndrome With Asplenia - p. 647 133. Mitral Atresia With Double-Outlet Right Ventricle and L-Loop Ventricles in the Heterotaxy Syndrome With Asplenia - p. 648 134. Alignment Concordance and Discordance Versus Situs Concordance and Discordance - p. 658 135. Occlusion of the Mitral Orifice By A Large, Ball-Like Rhabdomyoma Resulting in Stillbirth - p. 659 136. Congenital Mitral Stenosis - p. 659 137. Anatomic Types of Congenital Mitral Stenosis - p. 661 138. Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome, That is, With Aortic Valvar Atresia or Severe Congenital Aortic Valvar Stenosis - p. 664 139. Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome and Ventricular Septal Defect(s) - p. 674 140. Multiple Congenital Anomalies - p. 680 141. Anatomic Types of Parachute Mitral Valve - p. 683 142. Revised Definition of Parachute Mitral Valve - p. 684 143. The Conus (Infundibulum) - p. 686 144. Trisomy - p. 690 145. Congenital Mitral Stenosis With Ebstein’s Anomaly of the Tricuspid Valve - p. 699 146. Dilated Cardiomyopathy - p. 705 147. Forms of Marfan Syndrome - p. 711 148. Congenital Mitral Regurgitation With Transposition of the Great Arteries - p. 711 149. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,L} - p. 712 150. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,A} - p. 712 151. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,L,L} - p. 713 152. Congenital Mitral Regurgitation With Aberrant Left Coronary Artery from the Pulmonary Artery - p. 713 153. Congenital Mitral Regurgitation With Hypertrophic Obstructive Cardiomyopathy - p. 715 154. Congenital Mitral Regurgitation With Tetralogy of Fallot - p. 716 155. Congenital Mitral Regurgitation With Single Left Ventricle - p. 717 156. Congenital Mitral Regurgitation With Congenital Aneurysm or Dysplasia of the Left Ventricle - p. 719 157. Hypertrophic and Hypoplastic Cardiomyopathy - p. 720 158. Congenital Mitral Regurgitation With Down Syndrome - p. 721 159. Congenital Mitral Regurgitation With Double-Outlet Right Ventricle - p. 722 160. Congenital Mitral Regurgitation With Left Ventricular Outflow Tract Obstruction - p. 722 161. Congenital Mitral Regurgitation With Duchenne Muscular Dystrophy - p. 723 162. Congenital Mitral Regurgitation With Large Secundum Atrial Septal Defects - p. 724 163. Congenital Mitral Regurgitation With Heterotaxy Syndrome and Polysplenia - p. 726 164. Congenital Mitral Regurgitation With Hypoplastic Left Heart Syndrome - p. 727 165. Congenital Absence of the Mitral Valve Leaflets, Also Known as Congenitally Unguarded Mitral Orifice - p. 728 166. Adherent Mitral Valve Resulting in Fibrous Subaortic Stenosis - p. 730 167. Mitral Dysplasia - p. 731 168. Mitral Valve Hypoplasia Resulting Hemodynamically in Congenital Mitral Stenosis - p. 733 169. Myxomatous Mitral Valve - p. 735 170. Infantile Hypertrophic Cardiomyopathy - p. 736 171. Congenital Mitral Valve Prolapse - p. 740 172. Isolated Mitral Valve Prolapse - p. 741 173. Nonisolated Mitral Valve Prolapse - p. 741 174. Anatomic Considerations - p. 745 175. 15. Infundibuloarterial Situs Equations: How Normally and Abnormally Related Great Arteries Are Built and the Importance of Infundibuloarterial Situs Concordance and Discordance - p. 750 176. 16. Ventricular Septal Defects - p. 767 177. Components of the Ventricular Septum - p. 767 178. Relative Frequencies of the Four Anatomic Types of Ventricular Septal Defect - p. 769 179. Limitations of These Statistics - p. 770 180. Ventricular Septal Defect With and Without Aortic Insufficiency - p. 770 181. Summary - p. 772 182. Ventral Septal Defects As Parts of A Conotruncal Malformation - p. 806 183. 17. Single Ventricle - p. 818 184. 18. Superoinferior Ventricles - p. 834 185. 19. Anomalous Infundibular Muscle Bundles: Also Known as Anomalous Muscle Bundles of the Right Ventricle and as Double-Chambered Right Ventricle - p. 848 186. 20. Tetralogy of Fallot - p. 852 187. 21. Absence of the Subpulmonary Infundibulum With Its Sequelae Has Been Misinterpreted as Common Aortopulmonary Trunk - p. 867 188. 22. Transposition of the Great Arteries - p. 877 189. 23. Double-Outlet Right Ventricle - p. 916 190. 24. Double-Outlet Left Ventricle - p. 932 191. 25. Anatomically Corrected Malposition of the Great Arteries - p. 948 192. 26. What Prevents and What Permits the Embryonic Great Arterial Switch? - p. 956 193. 27. Infundibuloarterial Situs Equations and Analysis - p. 969 194. 28. The Cardiac Conduction System - p. 976 195. 29. The Heterotaxy Syndromes: Asplenia, Polysplenia, and With Normally Formed but Right-Sided Spleen - p. 989 196. What are the Heterotaxy Syndromes? - p. 989 197. What was the Atrial Situs? - p. 990 198. What structures are Involved? - p. 990 199. What is the Morphologically Right Atrium? - p. 990 200. What is the Morphologically Left Atrium? - p. 991 201. When can we not Diagnose the Atrial Situs? - p. 991 202. What Does Double-Outlet Right Ventricle With Asplenia Look Like? - p. 994 203. What Does the Heart in the Heterotaxy Syndrome With Polysplenia Look Like? - p. 1009 204. Relations Between the Great Arteries - p. 1018 205. Types of Atrial Situs - p. 1019 206. Literature - p. 1024 207. Management - p. 1025 208. Summary - p. 1025 209. 30. Conclusions - p. 1030 210. Appendix 1. Understanding Normally and Abnormally Related Great Arteries - p. 1033 211. Post Scriptum - p. 1048 212. E-Appendix 2. Chronological List of Publications 1961 to 2021 - p. 1048.e1 213. Index - p. 1049
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.
1. Cover image - p. i 2. Title page - p. ii 3. Table of Contents - p. iii 4. Copyright - p. iv 5. Dedication - p. v 6. Acknowledgments - p. vii 7. Section I. Historical Perspective - p. vii 8. 1. Brief History of the Cardiovascular System - p. 2 9. Section II. Cardiovascular Development - p. 11 10. 2. Embryology and Etiology - p. 14 11. Section III. Anatomic and Developmental Approach to Diagnosis - p. 58 12. 3. Morphologic Anatomy - p. 60 13. 4. Segmental Anatomy - p. 89 14. Section IV. Congenital Heart Disease - p. 104 15. 5. The Congenital Cardiac Pathology Database - p. 106 16. 6. Systemic Venous Anomalies - p. 113 17. Persistent Left or Right Superior Vena Cava - p. 113 18. Interruption of the Inferior Vena Cava - p. 123 19. Atresia or Stenosis of Coronary Sinus Ostium - p. 126 20. Aneurysms of the Sinus Venosus - p. 130 21. Absence or Atresia of the Right Superior Vena Cava - p. 133 22. Absence of Left Innominate Vein - p. 135 23. Innominate Vein Anterior to the Thymus - p. 136 24. The Raghib Syndrome - p. 137 25. Right Superior Vena Cava Draining Partly into the Left Atrium - p. 138 26. Retroaortic Innominate Vein - p. 140 27. Left-To-Right Switching of the Inferior Vena Cava - p. 142 28. Umbilical Vein to Coronary Sinus - p. 142 29. Anomalous “Portal” Vein to Azygos Vein - p. 142 30. Inferior Vena Cava Never Connects Directly With Left Atrium - p. 144 31. Summary - p. 151 32. 7. Pulmonary Venous Anomalies - p. 155 33. Totally Anomalous Pulmonary Venous Connection/Drainage - p. 155 34. Obstruction - p. 156 35. Classification of Totally Anomalous Pulmonary Venous Connection - p. 166 36. Findings - p. 172 37. Partially Anomalous Pulmonary Venous Connection/Drainage - p. 177 38. Discussion of Partially Anomalous Pulmonary Venous Connection and Partially Anomalous Pulmonary Venous Drainage - p. 192 39. Discussion and Literature Review on Partially Anomalous Pulmonary Venous Connection and Drainage - p. 194 40. Pulmonary Arteriovenous Fistulae - p. 206 41. Stenosis of the Pulmonary Veins - p. 209 42. Pulmonary Veno-Occlusive Disease - p. 216 43. Misalignment of Pulmonary Veins With Alveolar Capillary Dysplasia - p. 222 44. Pulmonary Varix - p. 222 45. Normal Anatomic Variations of the Pulmonary Veins - p. 222 46. Recent Investigations Concerning the Development of the Pulmonary Veins - p. 223 47. 8. Cor Triatriatum Sinistrum (Subdivided Left Atrium) and Cor Triatriatum Dextrum (Subdivided Right Atrium) - p. 234 48. 9. Interatrial Communications - p. 266 49. How Many Anatomic Types of Interatrial Communications Are There? - p. 266 50. Ostium Secundum Type of Atrial Septal Defect - p. 266 51. How Important Clinically Was Secundum Atrial Septal Defect II? - p. 278 52. Device Closure of Secundum Atrial Septum Defects - p. 298 53. Literature - p. 300 54. Pathologic Anatomy - p. 302 55. Diagnosis of Secundum Atrial Septal Defect - p. 306 56. Surgery for Secundum Atrial Septal Defect - p. 307 57. Transcatheter Intervention for Device Closure of Atrial Septal Defect Ii - p. 307 58. Summary - p. 309 59. 10. Juxtaposition of the Atrial Appendages - p. 320 60. 11. Common Atrioventricular Canal - p. 352 61. Morphology and Classification - p. 353 62. Embryology - p. 366 63. Etiology - p. 369 64. Parachute Mitral Valve - p. 370 65. Double-Orifice Mitral Valve - p. 374 66. Findings - p. 376 67. Ventriculoarterial Alignments - p. 377 68. Conus - p. 378 69. The Ventricular Septal Defects - p. 383 70. The Pulmonary Outflow Tract - p. 384 71. Partial Forms of Common AV Canal - p. 386 72. Segmental Anatomy - p. 387 73. Comparison of Pulmonary Valvar Obstruction - p. 390 74. Transitional Form of Common AV Canal - p. 391 75. Ventricular Septal Defect of the AV Canal Type - p. 391 76. Right Ventricle - p. 392 77. Left Ventricle - p. 393 78. Common AV Canal With Single Ventricle - p. 394 79. Findings - p. 395 80. Atrial Septum - p. 395 81. Ventricular Septum - p. 395 82. Segmental Anatomy - p. 397 83. Systemic Veins - p. 398 84. Pulmonary Veins - p. 398 85. The Atria - p. 399 86. Isolated Cleft of the Mitral Valve - p. 401 87. Discussion - p. 401 88. Summary - p. 406 89. 12. Double-Outlet and Common-Outlet Right Atrium - p. 416 90. 13. Tricuspid Valve Anomalies - p. 429 91. Ebstein’s Malformation - p. 429 92. Study of Ebstein’s Anomaly of the Tricuspid Valve and Right Ventricle - p. 435 93. Non-Ebstein Tricuspid Regurgitation - p. 462 94. Summary - p. 484 95. Tricuspid Atresia - p. 485 96. Findings - p. 488 97. Anatomic Details - p. 490 98. Arrhythmias - p. 518 99. Phylogeny - p. 519 100. Congenital Tricuspid Stenosis - p. 524 101. AV Discordance With VA Concordance - p. 533 102. Discussion - p. 543 103. Summary - p. 546 104. Our Series - p. 548 105. Patent Foramen Ovale - p. 548 106. Pathology - p. 553 107. Discussion - p. 563 108. Multiple Fenestrations (“Filigree”) Tricuspid Valve - p. 565 109. Literature - p. 566 110. Chapter Summary - p. 575 111. 14. Mitral Valve Anomalies - p. 594 112. Mitral Atresia - p. 594 113. Mitral Atresia With Normal Segmental Anatomy {S,D,S} - p. 597 114. Mitral Atresia With Normal Segmental Anatomy {S,D,S}, Intact Ventricular Septum, and Aortic Valvular Atresia - p. 597 115. Morphogenesis - p. 606 116. Mitral Atresia {S,D,S}, Intact Ventricular Septum, and Patent Aortic Valve - p. 608 117. Mitral Atresia {S,D,S}, Ventricular Septal Defect(s), and Patent Aortic Valve - p. 609 118. Mitral Atresia {S,D,S}, Ventricular Septal Defect, and Aortic Valvar Atresia - p. 614 119. Mitral Atresia {S,D,S}, Ventral Septal Defect, and Truncus Arteriosus - p. 615 120. 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 - p. 615 121. Mitral Atresia {S,D,S}, Aortic Valvar Atresia, Tricuspid Atresia, and Pulmonary Valvar Aresia in A Conjoined Twin - p. 628 122. Mitral Atresia With Double-Outlet Right Ventricle or Transposition of the Great Arteries in Visceroatrial Situs Solitus With Concordant D-Loop Ventricles - p. 632 123. Mitral Atresia With Ventricular Septal Defect and Double-Outlet Right Ventricle {S,D,D/“S”} - p. 632 124. Mitral Atresia With No Ventricular Septal Defect and Double-Oulet Right Ventricle {S,D,D} - p. 636 125. 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) - p. 640 126. Mitral Atresia (Right-Sided) With Ventricual Septal Defect and Double-Outlet Right Ventricle {S, L, L} (n = 4, 2.26% of the Series) - p. 641 127. Mitral Atresia (Right-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {S,L,L} - p. 643 128. Anatomic Type 13 With Mitral Atresia (Right-Sided), Intact Ventricular Septum, and Double-Outlet Right Ventricle {I,L,L} - p. 643 129. Mitral Atresia (Left-Sided) With or Without A Ventral Septal Defect and Double-Outlet Right Ventricle {I,D,D} - p. 645 130. Mitral Atresia (Left-Sided) With Intact Ventricular Septum and Transposition of the Great Arteries {I,D,D/A} - p. 646 131. Mitral Atresia With Double-Outlet Right Ventricle {A(S),D,D} and VISCERAL HETEROTAXY - p. 647 132. Mitral Atresia With Double-Outlet Right Ventricle and D-Loop Ventricles in the Heterotaxy Syndrome With Asplenia - p. 647 133. Mitral Atresia With Double-Outlet Right Ventricle and L-Loop Ventricles in the Heterotaxy Syndrome With Asplenia - p. 648 134. Alignment Concordance and Discordance Versus Situs Concordance and Discordance - p. 658 135. Occlusion of the Mitral Orifice By A Large, Ball-Like Rhabdomyoma Resulting in Stillbirth - p. 659 136. Congenital Mitral Stenosis - p. 659 137. Anatomic Types of Congenital Mitral Stenosis - p. 661 138. Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome, That is, With Aortic Valvar Atresia or Severe Congenital Aortic Valvar Stenosis - p. 664 139. Congenital Mitral Stenosis With Hypoplastic Left Heart Syndrome and Ventricular Septal Defect(s) - p. 674 140. Multiple Congenital Anomalies - p. 680 141. Anatomic Types of Parachute Mitral Valve - p. 683 142. Revised Definition of Parachute Mitral Valve - p. 684 143. The Conus (Infundibulum) - p. 686 144. Trisomy - p. 690 145. Congenital Mitral Stenosis With Ebstein’s Anomaly of the Tricuspid Valve - p. 699 146. Dilated Cardiomyopathy - p. 705 147. Forms of Marfan Syndrome - p. 711 148. Congenital Mitral Regurgitation With Transposition of the Great Arteries - p. 711 149. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,L} - p. 712 150. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,D,A} - p. 712 151. Congenital Mitral Regurgitation With Transposition of the Great Arteries {S,L,L} - p. 713 152. Congenital Mitral Regurgitation With Aberrant Left Coronary Artery from the Pulmonary Artery - p. 713 153. Congenital Mitral Regurgitation With Hypertrophic Obstructive Cardiomyopathy - p. 715 154. Congenital Mitral Regurgitation With Tetralogy of Fallot - p. 716 155. Congenital Mitral Regurgitation With Single Left Ventricle - p. 717 156. Congenital Mitral Regurgitation With Congenital Aneurysm or Dysplasia of the Left Ventricle - p. 719 157. Hypertrophic and Hypoplastic Cardiomyopathy - p. 720 158. Congenital Mitral Regurgitation With Down Syndrome - p. 721 159. Congenital Mitral Regurgitation With Double-Outlet Right Ventricle - p. 722 160. Congenital Mitral Regurgitation With Left Ventricular Outflow Tract Obstruction - p. 722 161. Congenital Mitral Regurgitation With Duchenne Muscular Dystrophy - p. 723 162. Congenital Mitral Regurgitation With Large Secundum Atrial Septal Defects - p. 724 163. Congenital Mitral Regurgitation With Heterotaxy Syndrome and Polysplenia - p. 726 164. Congenital Mitral Regurgitation With Hypoplastic Left Heart Syndrome - p. 727 165. Congenital Absence of the Mitral Valve Leaflets, Also Known as Congenitally Unguarded Mitral Orifice - p. 728 166. Adherent Mitral Valve Resulting in Fibrous Subaortic Stenosis - p. 730 167. Mitral Dysplasia - p. 731 168. Mitral Valve Hypoplasia Resulting Hemodynamically in Congenital Mitral Stenosis - p. 733 169. Myxomatous Mitral Valve - p. 735 170. Infantile Hypertrophic Cardiomyopathy - p. 736 171. Congenital Mitral Valve Prolapse - p. 740 172. Isolated Mitral Valve Prolapse - p. 741 173. Nonisolated Mitral Valve Prolapse - p. 741 174. Anatomic Considerations - p. 745 175. 15. Infundibuloarterial Situs Equations: How Normally and Abnormally Related Great Arteries Are Built and the Importance of Infundibuloarterial Situs Concordance and Discordance - p. 750 176. 16. Ventricular Septal Defects - p. 767 177. Components of the Ventricular Septum - p. 767 178. Relative Frequencies of the Four Anatomic Types of Ventricular Septal Defect - p. 769 179. Limitations of These Statistics - p. 770 180. Ventricular Septal Defect With and Without Aortic Insufficiency - p. 770 181. Summary - p. 772 182. Ventral Septal Defects As Parts of A Conotruncal Malformation - p. 806 183. 17. Single Ventricle - p. 818 184. 18. Superoinferior Ventricles - p. 834 185. 19. Anomalous Infundibular Muscle Bundles: Also Known as Anomalous Muscle Bundles of the Right Ventricle and as Double-Chambered Right Ventricle - p. 848 186. 20. Tetralogy of Fallot - p. 852 187. 21. Absence of the Subpulmonary Infundibulum With Its Sequelae Has Been Misinterpreted as Common Aortopulmonary Trunk - p. 867 188. 22. Transposition of the Great Arteries - p. 877 189. 23. Double-Outlet Right Ventricle - p. 916 190. 24. Double-Outlet Left Ventricle - p. 932 191. 25. Anatomically Corrected Malposition of the Great Arteries - p. 948 192. 26. What Prevents and What Permits the Embryonic Great Arterial Switch? - p. 956 193. 27. Infundibuloarterial Situs Equations and Analysis - p. 969 194. 28. The Cardiac Conduction System - p. 976 195. 29. The Heterotaxy Syndromes: Asplenia, Polysplenia, and With Normally Formed but Right-Sided Spleen - p. 989 196. What are the Heterotaxy Syndromes? - p. 989 197. What was the Atrial Situs? - p. 990 198. What structures are Involved? - p. 990 199. What is the Morphologically Right Atrium? - p. 990 200. What is the Morphologically Left Atrium? - p. 991 201. When can we not Diagnose the Atrial Situs? - p. 991 202. What Does Double-Outlet Right Ventricle With Asplenia Look Like? - p. 994 203. What Does the Heart in the Heterotaxy Syndrome With Polysplenia Look Like? - p. 1009 204. Relations Between the Great Arteries - p. 1018 205. Types of Atrial Situs - p. 1019 206. Literature - p. 1024 207. Management - p. 1025 208. Summary - p. 1025 209. 30. Conclusions - p. 1030 210. Appendix 1. Understanding Normally and Abnormally Related Great Arteries - p. 1033 211. Post Scriptum - p. 1048 212. E-Appendix 2. Chronological List of Publications 1961 to 2021 - p. 1048.e1 213. Index - p. 1049
