hd20heart2.ppt
TRANSCRIPT
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Human Embryology:Heart Development II
Human Embryology:Heart Development II
Kimara L. Targoff, M.D.Division of Pediatric Cardiology, Columbia University Medical Center
Developmental Genetics Program, Skirball Institute, NYU School of Medicine
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Human Vascular Development
• Overview• Aortic Arch Development• Arterial Vascular Development• Venous System Development• Lymphatic Development• Transition from Fetal to Post-Natal Circulation
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Development of the Arterial and Venous Systems
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Cranial Ends of the Dorsal Aortae Form a Dorsoventral Loop: The First Aortic Arch
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Aortic Arches Arise in a Craniocaudal Sequence Surrounding the Pharynx
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Aortic Arches Give Rise to Important Head, Neck, and Upper Thorax Vessels
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Aortic Arch Development in the Chick Embryo
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Fgf8 is Required for Pharyngeal Arch Development in Mouse
Abu-Issa, R. et al., Development 2002.
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Cardiovascular and Thymic Defects in Tbx1 Hypomorphic Mutant Neonates
Hu, T. et al., Development 2004.
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Aortic Arch Development
12
3
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
Harsh Thaker
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Aortic Arch Development
12
3
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
Harsh Thaker
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6
33
44
7 iseg7 iseg
Aortic sac
Truncus arteriosus
6
Aortic Arch and Derivatives
Harsh Thaker
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6
33
44
7 iseg7 iseg
Harsh Thaker
Aortic Arch and Derivatives
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6
33
4
47 iseg
7 iseg
Aortic Arch and Derivatives
Harsh Thaker
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LCCRCC
RSC
BCA
LSC
DA
Aortic Arch and Derivatives
Harsh Thaker
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LCCRCC
RSC
BCA
LSC
DA
Recurrent Laryngeal Nerves
Harsh Thaker
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Defects in Normal Regression of the Arterial System Lead to Vascular Anomalies
• Double Aortic Arch – Failure of the right dorsal aorta to regress
• Aberrant Right Subclavian Artery– Regression of the right fourth arch– 1% of the general population– 40% of patients with Trisomy 21 and CHD
• Right Aortic Arch– Retention of the right dorsal aorta segment– 13-35% of patients with TOF– 8% of patients with TGA
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Failure of Regression of the Right Dorsal Aorta Leads to a Double Aortic Arch
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Double Aortic Arch
12
3
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
Harsh Thaker
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Regression of the Right Fourth Arch Results in an Aberrant Right Subclavian Artery
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Aberrant Right Subclavian Artery
12
3
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
Harsh Thaker
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LCCRCC
RSC
BCA
LSC
DA
Harsh Thaker
Aberrant Right Subclavian Artery
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Retention of the Right Dorsal Aortic Segment Yields a Right Aortic Arch
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Right Aortic Arch
12
3
6
4
5
7 iseg
Ventralaorta
Dorsal aorta
Harsh Thaker
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Right Aortic Arch: Mirror Image Branching versus Aberrant Left Subclavian Artery
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Vascular Rings May Cause Compression of the Trachea and the Esophagus
• Double Aortic Arch– Failure of the right dorsal aorta to regress
• Right Aortic Arch– Ductus arteriosus is directed towards the right– If the ductus, or later, the ligamentum arteriosum, passes behind
the esophagus, constriction may occur
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Double Aortic Arch Presenting with Dysphagia in a 31-Year-Old Woman
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Aortic Arch Anomalies Can Cause Significant Clinically Compromise in the Neonatal Period
• Interrupted Aortic Arch– Obliteration of the right and left fourth aortic arches
• Coarctation of the Aorta– Constriction of the aorta in the region of the ductus arteriosus– 0.3% of live births– Most common cardiac anomaly in Turner’s Syndrome
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Obliteration
of the
Right
and
Left
Fourth
Aortic
Arches
Leads to
an
Aortic
Arch Interruption
Obliteration of the Right and Left Fourth Aortic Arches Leads to Interruption of the Aorta
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Constriction of the Aorta in the Region of the Ductus Arteriosus Produces Coarctation
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Post-ductal Coarctation of the Aorta Utilizes Collateral Circulation to Supply Blood to the Lower Body
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Post-ductal Coarctation of the Aorta Utilizes Collateral Circulation to Supply Blood to the Lower Body
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Vitelline Arteries Give Rise to the Arterial Supply of the Gastrointestinal Tract
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Lateral Branches of the Descending Aorta Highlight Developmental Histories of Each Organ
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Umbilical
Vitelline
The Developing Venous System
SinusVenosus
Cardinal
Harsh Thaker
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Vitelline Veins Form a Portal System to Drain Blood from the Foregut, Midgut, and Part of the Anorectal Canal
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Umbilical
Vitelline
The Developing Venous System
SinusVenosus
Cardinal
Harsh Thaker
Subcardinal
Supra cardinal
Supra-SubcardinalAnastomosis
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The Systemic Venous System Develops from Four Bilaterally Symmetric Cardinal Veins
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Following Remodeling of the Subcardinal System, the Supracardinal Veins Sprout
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Remodeling of Abdominal Venous System Occurs through Obliteration of the Left Supracardinal Vein
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Failure of Left Cardinal Veins to Undergo Normal Regression Leads to Venous Anomalies
• LSVC occurs in 0.3% to 0.5% of the normal population
• In 65% of cases, left brachiocephalic vein is also missing
• 4% of patients with CHD have an LSVC
• Usually drains to the coronary sinus
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Lymph Sacs and Ducts Form by Lymphangiogenesis to Drain Fluid from Tissue Spaces Throughout the Body
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Cystic Hygromas Develop in Turner’s Syndrome Patients Secondary to Blockage of Lymphatic Ducts
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Fetal Circulation Bypasses the Developing Pulmonary Circulation
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Pulmonary Vascular Resistance Drops Precipitously and Initiates the Transition to Post-Natal Circulation
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Normal Closure of the Ductus Arteriosus Occurs during the Transition to Neonatal Circulation in Series
• Prostaglandins maintain a patent ductus arteriosus• Indomethacin is used to induce ductal closure• Physiologic closure occurs by 2 days in 82% of patients