마더세이프 라운드 - principles of embryology (전이경 교수
TRANSCRIPT
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Cardiac Development
관동의대 제일병원병리과 전이경
2010. 6. 15
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Congenital Heart DiseaseFrequency 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
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Critical periods of development for various organ systems and the resultant malformations
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First week of development: Ovulation to implantation
Second week of development:Bilaminar germ disc
Third week of development: Gastrulation (trilaminar germ disc)
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Truncus
Conus
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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
day 18
day 22
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A, day 18. B, day 20. C, day 21. D, day 22
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Early Cardiac Morphogenesis II(Fertilization 4th week)
• Segmentation: SV-AT-AVC- VT-BC
• Layering: Endocardium – Myocardium- Epicardium
Cardiac jelly• Looping
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AtriumBulbus cordis
VentricleSinus
venosusAV canal
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A, day 22 B, day 23 C, day 24
Formation of Cardiac loop
Normal D-looping
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Abnormalities of cardiac looping
• Dextrocardia:• 심장이오른쪽흉곽에위치하고심첨이오른쪽을향함
• Isolated form/ situs inversus
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Formation of cardiac septa
• Between 27th and 37th days of development (embryo: 5mm~ 16-17mm)
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Atrial Septation (4-6 week)
• Primary septum (septum primum)• Secondary septum (septum secundum)• Intermediate septum (septum
intermedium)- endocardial cushion
• Primary foramen (Foramen primum) • Secondary foramen (Foramen secundum) • Oval foramen (foramen oval)
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Atrial Septal Defect
• TypeSecundum ASD (90%)Primum ASD (5%)Sinus venosus ASD (5%)
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Secundum ASD (90%)
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Endocardial cushion
• Atrioventricular and conotruncal regions 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. TGA,
TOF)
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Ventricular Septation
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35 days
30 days
7 weeks
Development of conotruncal ridges and closure of the interventricular
foramen
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Development of conotruncal ridges and closure of the interventricular foramen
6 weeks Beginning of 7 weeks
End of 7 weeks
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Ventricular Septation (5-8 week)• Muscular ventricular septum • Expansion of ventricles• Closure of interventricular foramen
– Interventricular septum – Atrioventricular ECC– Outflow tract endocardial ridge
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Ventricular Septal Defect• Type
perimembranous (80-90%)muscular (5-20%)outlet or infundibular, (doubly committed) juxta-arterial (5-7%)
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Perimembranous inlet extensiontype
Muscular type
(Doubly committed) juxta-arterial typeInfundibular typeOutlet type
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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
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• Essential defect : Anterosuperior displacement of outlet septum
PA
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Persistent truncus arteriosus
• Conotruncal ridge– fail to fuse and to
descend toward the ventricles
– interventricular septum형성에도 관여하므로 언제나interventricular septal defect동반
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PTAO
LV
RV
Conotruncal septum → fail to follow its normal spiral course and run
straight down → Aorta from RV, PA from LVVSD, perimembranous type 동반하기도.
Transposition of Great Arteries
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Neural crest cells
• Crest cells– Vulnerable cell population – Deficient in superoxide dismutase and catalase enzymes
that are responsible for scavenging free radicals.→ Easily killed by compounds such as alcohol and retinoic acid.
• Essential for formation of much of the cranial region– Disruption of crest cell development ☞ Severe craniofacial malformations☞ Examples: Treacher-Collins' Syndrome, DiGeorge anomaly, Robin sequence….
• Conotruncal endocardial cushions☞ cardiac anomalies including persistent truncus arteriosus,
TOF and TGA.
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Velocardiofacial Syndrome/DiGeorge anomaly
• 22q11.2 deletion• “CATCH 22”
– Cardiac defects– Abnormal face– Thymic hypoplasia – Cleft palate– Hypocalcemia
• Abnormal development of neural crest cells
• Specific facial features – low-set ears, wide-set
eyes, a small jaw, and a short groove in the upper lip
• Etiology– Genetic causes, exposure
to retinoic acids, alcohol, and maternal DM
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Formation of Atrioventricular Valve
• Endocardial cushion + Ventricular myocardium• Undermining of myocardium
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Formation of Ventriculoarterial Valve
5 weeks
6 weeks
7 weeks
6 weeks
7 weeks 9 weeks
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Development of Conduction System
• Cardiomyogenic origin of conduction cells
• Emergence of cardiac pacemaker in embryonic tube heart
• Development of atrioventricular delay in looping, tube heart
• Differentiation of fast conduction system during chamber septation
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Left-right Sidedness and Heart
• First organ with asymmetry• Genetic difference between right and
left before morphologic asymmetry• LA and RA - left-right sidedness • LV and RV - anteroposterior axis
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PITX2: a transcription factor responsible for establishing left sidedness
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Subdivision of Primary Heart TubeHeart tube Looped heart Adult heart
truncus arteriosus Great arteriesOFT
conus cordis
Bulbus cordis
Embryonic right ventricle
RV
Ventricle Embryonic left ventricle LV
Atrioventricular canal (AVC)
Atrium Atrium Atrium
Sinus venosus Sinus venosus
Caval veins
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4-mm embryo (end of the fourth week)
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Venous system
• Vitelline system -> portal system• Cardinal system -> caval system• Umbilical system -> disappear after
birth
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Double aortic arch
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Abnormal origin of the right subclavian artery
Interruption of aortic arch
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IVC
Normal heart