심장의 발생   2012. 11. 21 관동의대 제일병원  병리과 전이경
심장의 발생•   embryo에서 최초로 기능을 하는 장기•   수정 4 주 초 박동 시작•   발생: 수정 후 3주- 8주•   선천성 심장병: 가장 흔한 선천성 기형
Critical periods of development for various organ     systems and the resultant malformations
Congenital Heart Disease                         Frequency of Congenital Cardiac Malformations                       Malfo...
First week of development: Ovulation to implantation
Second week of development:        Bilaminar germ discday12                       day13
Third week of development:Gastrulation (trilaminar germ disc)   day16
20 days Neurulation includesthe formation of theneural plate (day 18-19),neural folds (day 20-21),and the neural tube (day...
Final closureAnterior neuropore: 25th dayPosterior neuropore: 27th day
day 18
Heart tubeA, day 18B, day 20C, day 21D, day 22*
Early Cardiac Morphogenesis I       (Fertilization 3rd week)• Cardiogenic crest in  splanchnic mesoderm in  front of the n...
Early Cardiac Morphogenesis II     (Fertilization 4th week)• Segmentation  : Sinus Venosus- Atrium     - AV canal- Ventric...
Formation of Cardiac loop         Normal D-loopingday 22        day 23        day 24
Cardiac Segmentation, Layering, Looping
30 days
Abnormalities of Cardiac Looping• Dextrocardia  – Isolated form  – Situs inversus• Heterotaxy (Laterality  sequences)  – P...
Development of sinus venosus                                                             Fetal stageACV: anterior cardinal...
Formation of cardiac septa
Atrial Septation (4-6 weeks)             Septum primum      ostium primum                             Interventricular    ...
ostiumsecundum                      35 days           Septum           secundum
Atrial Septum•   Primary septum (septum primum)•   Secondary septum (septum    secundum)•   Intermediate septum (septum   ...
Secundum ASD (90%)
Endocardial cushion•   Atrioventricular canal and conotruncal region    •     Atrial and ventricular (membranous portion) ...
Neural crest cells in conotruncal region
35 days
Development of conotruncal ridges andclosure of the interventricular foramen6 weeks                       Beginning of 7 w...
End of 7 weeks
7 weeks
Ventricular Septation (4주 말-8주)• Muscular interventricular septum + Membranous  part of interventricular septum• Closure o...
Formation of Ventriculoarterial Valve                       5 weeks   6 weeks       7 weeks             6 weeks   7 weeks ...
Ventricular Septal Defect• Most common congenital  cardiac malformation• Isolated or a/w  abnormalities in  partitioning o...
Perimembranous type                      Muscular type                            (Doubly committed) juxta-arterial type  ...
Tetralogy of Fallot• Most common cyanotic CHD• Conotruncal region 이상   • Unequal division of the conus   resulting from an...
Persistent truncus arteriosus• Conotruncal ridge  – fail to fuse and to    descend toward the    ventricles  – interventri...
Transposition of Great Arteries                                             AO                                            ...
경청해주셔서 감사합니다☺
Human Cardiac embryology - 전이경 교수
Human Cardiac embryology - 전이경 교수
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Human Cardiac embryology - 전이경 교수

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Human Cardiac embryology - 전이경 교수

  1. 1. 심장의 발생 2012. 11. 21 관동의대 제일병원 병리과 전이경
  2. 2. 심장의 발생• embryo에서 최초로 기능을 하는 장기• 수정 4 주 초 박동 시작• 발생: 수정 후 3주- 8주• 선천성 심장병: 가장 흔한 선천성 기형
  3. 3. Critical periods of development for various organ systems and the resultant malformations
  4. 4. Congenital Heart Disease Frequency of Congenital Cardiac Malformations Malformation Incidence/Million Live Births %Ventricular septal defect (VSD) 4482 42Atrial septal defect (ASD) 1043 10Pulmonary stenosis (PS) 836 8Patent ductus arteriosus (PDA) 781 7Tetralogy of Fallot (TOF) 577 5Coarctation of aorta (CoA) 492 5Atrioventricular septal defect (AVSD) 396 4Aortic stenosis (AS) 388 4Transposition of great arteries (TGA) 388 4Truncus arteriosus 136 1Total anomalous pulmonary venous connection (TAPVC) 120 1Total 9757 J Am Coll Cardiol 39:1890,2002
  5. 5. First week of development: Ovulation to implantation
  6. 6. Second week of development: Bilaminar germ discday12 day13
  7. 7. Third week of development:Gastrulation (trilaminar germ disc) day16
  8. 8. 20 days Neurulation includesthe formation of theneural plate (day 18-19),neural folds (day 20-21),and the neural tube (day22-26); the latter willdevelop into the futurebrain and spinal cord
  9. 9. Final closureAnterior neuropore: 25th dayPosterior neuropore: 27th day
  10. 10. day 18
  11. 11. Heart tubeA, day 18B, day 20C, day 21D, day 22*
  12. 12. Early Cardiac Morphogenesis I (Fertilization 3rd week)• Cardiogenic crest in splanchnic mesoderm in front of the neural plate• Bilateral endocardial tube from angioblastic cords• Cephalic and lateral folding of the embryo• Primary heart tube in the middle thorax
  13. 13. Early Cardiac Morphogenesis II (Fertilization 4th week)• Segmentation : Sinus Venosus- Atrium - AV canal- Ventricle - Bulbus Cordis• Layering Ventricle Atrium – Endocardium Bulbus cordis AV Sinus – Myocardium canal venosus – Epicardium – Cardiac jelly• Looping
  14. 14. Formation of Cardiac loop Normal D-loopingday 22 day 23 day 24
  15. 15. Cardiac Segmentation, Layering, Looping
  16. 16. 30 days
  17. 17. Abnormalities of Cardiac Looping• Dextrocardia – Isolated form – Situs inversus• Heterotaxy (Laterality sequences) – Polysplenia syndrome- left isomerism – Asplenia syndrome- right isomerism
  18. 18. Development of sinus venosus Fetal stageACV: anterior cardinal v. PCV: posterior cardinal v.UV: umbilical v. VIT: vitelline v. CCV: common cardinal v.
  19. 19. Formation of cardiac septa
  20. 20. Atrial Septation (4-6 weeks) Septum primum ostium primum Interventricular foramen
  21. 21. ostiumsecundum 35 days Septum secundum
  22. 22. Atrial Septum• Primary septum (septum primum)• Secondary septum (septum secundum)• Intermediate septum (septum intermedium)- endocardial cushion• Primary foramen (osteum primum)• Secondary foramen (osteum secundum)• Oval foramen (foramen ovale)
  23. 23. Secundum ASD (90%)
  24. 24. Endocardial cushion• Atrioventricular canal and conotruncal region • Atrial and ventricular (membranous portion) septa • Atrioventricular canals and valves • Aortic and pulmonary channels• Abnormalities in ECC formation • ASD, VSD, AVSD • Defects involving great vessels (ex. persistent truncus arteriosus, TOF and TGA)
  25. 25. Neural crest cells in conotruncal region
  26. 26. 35 days
  27. 27. Development of conotruncal ridges andclosure of the interventricular foramen6 weeks Beginning of 7 weeks
  28. 28. End of 7 weeks
  29. 29. 7 weeks
  30. 30. Ventricular Septation (4주 말-8주)• Muscular interventricular septum + Membranous part of interventricular septum• Closure of interventricular foramen – Interventricular septum – Atrioventricular ECC – Outflow tract endocardial ridge
  31. 31. Formation of Ventriculoarterial Valve 5 weeks 6 weeks 7 weeks 6 weeks 7 weeks 9 weeks
  32. 32. Ventricular Septal Defect• Most common congenital cardiac malformation• Isolated or a/w abnormalities in partitioning of the conotruncal region• Type – perimembranous (80-90%) – muscular (5-20%) – outlet or infundibular, (doubly committed) juxta- arterial (5-7%)
  33. 33. Perimembranous type Muscular type (Doubly committed) juxta-arterial type Infundibular type Outlet type
  34. 34. Tetralogy of Fallot• Most common cyanotic CHD• Conotruncal region 이상 • Unequal division of the conus resulting from anterior displacement of the conotruncal septum• Four features 1) Ventricular septal defect 2) Subpulmonary stenosis 3) Overriding of aorta 4) Right ventricular hypertrophy
  35. 35. Persistent truncus arteriosus• Conotruncal ridge – fail to fuse and to descend toward the ventricles – interventricular septum 형성에도 관여하므로 언제나 interventricular septal defect동반
  36. 36. Transposition of Great Arteries AO PT RV LVConotruncal septum→ fail to follow its normal spiral course and run straight down→ Aorta from RV, PA from LVVSD, perimembranous type 동반하기도.
  37. 37. 경청해주셔서 감사합니다☺

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