heart and its neighborhood neural crest cells migrate from hindbrain region via pharyngeal arches to...
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HEART AND ITS NEIGHBORHOOD
NEURAL CREST CELLS migratefrom hindbrain region via pharyngeal arches to conotruncal region of heart.
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BLOOD VESSELS OF THE EMBRYO (at 26 days)
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AORTIC ARCHES SCHEMATIC
1
23
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
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AORTIC ARCHES SCHEMATIC
3
6
4
12
5
7 iseg
Ventralaorta
Dorsal aorta
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6
33
44
7 iseg7 iseg
AORTIC ARCHES AND DERIVATIVES
Aortic sac
Truncus arteriosus
6
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6
33
44
7 iseg7 iseg
AORTIC ARCHES AND DERIVATIVES
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6
33
4
47 iseg
7 iseg
AORTIC ARCHES AND DERIVATIVES
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LCCRCC
RSC
BCA
LSC
DA
AORTIC ARCHES AND DERIVATIVES
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6
33
4
47 iseg
7 iseg
RECURRENT LARYNGEAL NERVES
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LCCRCC
RSC
BCA
LSC
DA
RECURRENT LARYNGEAL NERVES
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3
6
4
12
5
7 iseg
Ventralaorta
Dorsal aorta
DOUBLE AORTIC ARCH
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DOUBLE AORTIC ARCH: “Vascular ring”Causes airway obstruction, stridor in infancy.
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3
6
4
12
5
7 iseg
Ventralaorta
Dorsal aorta
RIGHT AORTIC ARCH
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RIGHT AORTIC ARCH: Mirror image branching
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3
6
4
12
5
7 iseg
Ventralaorta
Dorsal aorta
ABERRANT RIGHT SUBCLAVIAN ARTERY
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LCCRCC
RSCLSC
DA
ABERRANT RIGHT SUBCLAVIAN ARTERY
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ABERRANT RIGHT SUBCLAVIAN ARTERY
Occurs in 0.5% of people.Frequently asymptomatic, it may cause obstructive symptoms.
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COARCTATION OF THE AORTA
Notching of ribs on X-ray
Blood pressure differenceUpper extr >>Lower extr.
Associated with:45X: Turner’s synromeTrisomy 21: Down’s syndrome
70% have bicuspid aortic valve
>90% post (juxta) ductal
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ABDOMINAL ARTERIES
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Umbilical
Vitelline
VENOUS SYSTEM SCHEMATIC
SinusVenosus
Cardinal
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© 2005 Elsevier
UMBILICAL AND VITELLINE VEINS- I:Liver, portal vein and ductus venosus.
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UMBILICAL AND VITELLINE VEINS- II:Liver, portal vein and ductus venosus.
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Cardinal
Subcardinal
Supra cardinal
Supra-SubcardinalAnastomosis
Umbilical
Vitelline
VENOUS SYSTEM SCHEMATIC
SinusVenosus
C
P
A
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THE CARDINAL VEINS AND THE VENAE CAVAE
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THE CARDINAL VEINS AND THE VENAE CAVAE
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SINUS VENOSUS AND THE CORONARY SINUS
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VENOUS (SMOOTH WALLED) PART OF ATRIA
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PERSISTENT LEFT SVC
0.3% of general population.4 % of patients with Cong. Ht Dis.
Usually drains to Coronary sinus.Usually asymptomatic.
Enlarged coronary sinus is a clue.
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LYMPHATIC SYSTEM
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FETAL CIRCULATION
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POSTNATAL CIRCULATION
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PATENT DUCTUS ARTERIOSUS
Prostaglandins: Keep the ductus PatentIndomethacin: Closes the ductus.
Physiologic closure: Normally 82% by 48hrs, 100% by 4 days.Anatomic closure: 12 weeks.
Patent Ductus : -prematurity.-neonatal hypoxic states.-maternal Rubella infection
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CELL LINEAGES IN HEART DEVELOPMENT
FHF = First Heart Field; SHF = Second Heart Field; CNC: Cardiac Neural Crest
Ref: Srivastava, D. Making or Breaking the Heart: From Lineage Determination to Morphogenesis.Cell 126, Sep. 22, 2006 p1037-1048.
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MOLECULAR PATHWAYS IN HEART DEVELOPMENT
Ref: Srivastava, D. Making or Breaking the Heart: From Lineage Determination to Morphogenesis.Cell 126, Sep. 22, 2006 p1037-1048.
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Genetic Mutation
Syndrome Name
Cardiac Disease
Nonsyndromic
NKX2-5 —Atrial septal defect, ventricular septal defect, electrical conduction defect
GATA4 — Atrial septal defect, ventricular septal defect
MYH6 — Atrial septal defect
NOTCH1 — Aortic valve disease
Syndromic
TBX5 Holt-OramAtrial septal defect, ventricular septal defect, electrical conduction defect
TBX1 DiGeorge Cardiac outflow tract defect
TFAP2β Char Patent ductus arteriosus
JAG1 Alagille Pulmonary artery stenosis, tetralogy of Fallot
PTPN11 Noonan Pulmonary valve stenosis
Elastin William Supravalvar aortic stenosis
Fibrillin Marfan Aortic aneurysm
Genetic Mutations in Congenital Heart Disease
Ref: Srivastava, D. Making or Breaking the Heart: From Lineage Determination to Morphogenesis.Cell 126, Sep. 22, 2006 p1037-1048.
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MOVIE!