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Embryology of the Heart
For the puposes of narration, this is slide number 0.
Cardiogenic area derived fromIntra-embryonic mesoderm
- mid 3rd week
Figures: Sadler 2004
Two cardiogenic cordsdevelop within cardiogenic area. Cords become canalized to give rise to two endocardial heart tubes.
Figure: Larsen 1993
Figure: Sadler 2004
Figures: Sadler 2004
Late 3rd week
Figures: Sadler 2004
Late 3rd week
Figure: Larsen 1993
Endocardial heart tubes come to lieside by side ventral to foregut.
Figure: Larsen 1993
•At 21 days, two endocardial heart tubes begin to fuse into one tube. •Caudal end of heart tube in anchored in septum transversum.•Cranial end is developing connections to paired dorsal aortae. •Fusion of tubes is complete by 23 days.
Figure: Gilbert 1989
Figure: Moore&Persaud 1998
On day 22, tube begins to elongate, and develops alternate dilations and constrictions. Begin to see primitive heart chambers:
•Bulbus cordis•Ventricle•Atrium
Figure: Larsen 1993
•On day 23, common venous collection chamber added: sinus•venosus, and common origin for aortic arches: truncus arteriosus.•As heart tubes elongates, loop forms to right: bulboventricular loop.
Figures: Sadler 2004
Truncusarteriosus
Figure: Larsen 1993
Day 23
By day 22 or 23,contractions of myogenic origin begin. Occur in peristaltic waves beginning at venous end.
Figure: Sadler 2004
Day 24
Figure: Sadler 2004
Figure: Moore&Persaud 1998
Figure: Gilbert 1989
Figure: Larsen 1993
4 week heart
Figure: Sadler 2004
4 – 5 week heart
Figure: Sadler 2004
4 week heart
Externally, ventricle and atrium appear to have right and left sides.
Figure: Sadler 2004
Internally, there is still unidirectional blood flow: Sinus venosus RA LA Atrioventricular Canal LV RV(bulbus cordis) Conus cordis (bulbus cordis) Truncus arteriosus
4 week heart
Figure: Gilbert 1989
Late 4th week – swellings develop in dorsal and ventral walls of atrioventricular canal: AV endocardial cushions.
Figure: Gilbert 1989
Late 4th week:View from theright side.
Figure: Gilbert 1989
Figure: Gilbert 1989
During 5th weekendocardialcushions fuse todivide AV canalinto right andleft AV canals.
Figure: Gilbert 1989
Division of atriuminto right and leftatria also beginsin the 5th week.
Figure: Gilbert 1989
Division of atrium begins with the appearance of a crescent-shaped membrane that appears in roof and grows ventrally toward the endocardial cushtions: septum primum.
Figure: Gilbert 1989
The opening between the growing septum primum and the endocardial cushions, which allows blood flow from right to left, is called the foramen primum.
Figure: Gilbert 1989
As septum primum grows, foramen primum gets smaller eventually disappears. New foramen develops high up in septum: foramen secundum.
Figure: Gilbert 1989
Toward end of 5th week, new crescent-shaped ridge appears in roof of atrum to right of septum primum: septum secundum. More muscular than first.
Figure: Gilbert 1989
As septum secundum grows (into 6th week), covers foramen secundum. Opening made by inferior margin of septum secundum called foramen ovale. Foramen ovale persists throughout fetal development.
Figure: Gilbert 1989
Figure: Larsen 1993
Close approximation between opening of inferior vena cava and foramen ovale causes blood from the IVC to pass from RA to LA, and into LV and out aorta.
Figure: Larsen 1993
Because septum secundum is ratherrigid while septum primum is moreflexible, combination of septa andformena work as a unidirectional fluttervalve.
Figures: Gilbert 1989: Moore&Persaud 1998
Figures: Larsen 1993
As atrium is being divided, part of sinus venosus is drawn Into wall of RA giving rise to smooth-walled portion of RA known as the sinus venarum. Another part of sinus venosus is incorporated into the LA, and gives rise to a pulmonary vein, the stem of which is also drawn into wall of the LA.
Figure: Moore&Persaud 1998
Figure: Sadler 2004
Figure: Moore&Persaud 1998
Figure: Sadler 2004
Day 35
Completion of the interventricular septum is problematic because truncus arteriosus is a right sided structure, and blood from LV must pass through IV foramen to exit out the aorta.
Figure: Langebartel&Ullrich 1977
If the truncus had been positioned in the midline, then the ventricles could be separated by the spiral septum merging with the original IV septum.
Figure: Langebartel&Ullrich 1977
But since the truncus is on the right, the ventricles are separated by the addition of an oblique septum derived from bulbar ridges, truncal ridges, and endocardial cushions, that partition off part of the bulbus cordis to be incorporated into the LV as the aortic vestibule.
Figure: Langebartel&Ullrich 1977
To divide the ventricle while maintaining an outflow track for the LV, must divide the bulbus cordis so that part is incorporated into the LV for its outflow track.
Figures: FitzGerald 1978
Beginning in 5th week, bulbar ridges (neural crest cells) form in right posterior and left anterior walls of bulbus cordis. Endocardial cusion area also begins to proliferate. Figures: FitzGerald 1978
The septum created By the fused bulbar ridges fuses to the aorticopulmonary septum superiorly.
Inferiorly, the bulbar septum joins with original interventri-cular septum and with material from endocardial cushions to complete division of the ventricle.
Figure: FitzGerald 1978
Figure: Sadler 2004
Completion of Interventricular Septum
• Original IV septum becomes thick-walled portion of adult IV septum
• Part of septum derived from bulbar ridges, truncal ridges and endocardial cushions becomes membranous portion of adult IV septum
• Right anterior portion of bulbus cordis incorporated into RV as conus arteriosus or infundibulum
• Left posterior portion of bulbus cordis incorporated into LV as aortic vestibule
By 8 weeks all major components of heart development are complete
• As septa are developing, internal features such a trabeculae carneae within the ventricles are developing
• Atrioventricular valves form on R & L
• Semilunar valves form in aorta and pulmonary trunk
• Conducting system develops
Cardiac Malformations
• Because development is complex, malformations are relatively common– Approximately 20% of congenital
malformations involve heart and/or great arteries
– Estimated to occur in about 1% live births and ten times more frequently in still births (Sadler 2004).
• Following list are fairly common defects amenable to surgery
Atrial Septal Defects (ASD)
• Interatrial septum fails to form properly• More common in females than males
(3:1)• If large, will cause interatrial shunting of
blood and hypertrophy of RV and pulmonary trunk
• Large ASD: 6 out of 10,000 births (0.06%)
Atrial Septal Defects (ASD)
Figure: Moore&Persaud 1998
Atrial Septal Defects (ASD)
Incomplete fusion of septum primum and septum secundum large enough for a probe to pass through: Probe patent foramen ovale. Estimated to occur In about 25% of population.
Figure: Moore&Persaud 1998
Atrioventricular Septal Defect
Figure: Moore&Persaud 1998
Ventricular Septal Defect (VSD)
• Interventricular septum incomplete and fails to fully divide ventricles
• Most common of all congenital cardiac defects: – About 25% of cardiac abnormalities
documented in live births include VSDs– Isolated defects occur in 10-12 of 10,000
births (0.10%)– More common in males than females
•Most VSD occur in membranous portion of septum•Less commonly, VSD occur in muscular part of septum, probably due to excessive resorption of myocardial tissue
Figure: Sadler 2004
Normal complete interventricular septum
Figure: Sadler 2004
Figure: Moore&Persaud 1998; Larsen 1993
Dextrocardia
Abnormal Division of Truncus Arteriosus
Persistent truncus arteriosus•Failure of aorticopulmonary septum to form•Approximately 1 in 10,000 births (0.01%)•Necessarily includes VSD•Results in mixing of blood from R&L sides of heart•Can be fatal if untreated
Figure: Sadler 2004
Abnormal Division of Truncus Arteriosus
Transposition of Great Arteries•Aorticopulmonary septum does not spiral•Approx. 2 in 10,000 births (0.02%); more common in infants of diabetic mothers; more common in males than females (3:1)•Usually mixing of blood through patent foramen ovale or ductus arteriosus
Figure: Sadler 2004
Abnormal Division of Truncus Arteriosus
Unequal Division of Truncus Arteriosus•One artery large, other small (stenotic)•Ventricle on stenotic side must work harder, typically hypertrophies•Often aorticopulmonary septum not aligned with interventricular septum and also have VSD
Figure: Sadler 2004
Tetralogy of Fallot
Combination of defects, seen in approx. 10 in 10,000births (0.10%). 1. Pulmonary stenosis2. VSD3. Overriding aorta4. Hypertrophy of right ventricle
Figure: Sadler 2004
Figure References
• FitzGerald, M. J. T. (1977) Human Embryology: A Regional Approach. New York: Harper & Row Publ.
• Gilbert, S. G. (1989) Pictorial Human Embryology. Seattle: Univ. Washington Press
• Langebartel, D. A. & Ullrich, R. H. Jr. (1977) The Anatomical Primer: An Embryological Explanation of Human Gross Morphology. Baltimore: Univ. Park Press.
• Larsen, W. J. (1993) Human Embryology. New York: Churchill Livingstone.
• Moore, K. L. & Persaud, T. V. N. (1998) The Developing Human: Clinically Oriented Embryology. Philadelphia: W. B. Saunders Co.
• Sadler, T. W. (2004) Langman’s Medical Embryology, 9th Ed. Philadelphia: Lippincott Williams & Wilkins.