Endocardial cushion defect pathophysiology: Difference between revisions
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* Leading to heart failure and ↑ PVR | * Leading to heart failure and ↑ PVR | ||
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|'''Partial | |'''Partial defect''' | ||
|At the level of the primum atrial septal defect | |At the level of the primum atrial septal defect | ||
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* Minimal symptoms | * Minimal symptoms | ||
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|'''Transitional | |'''Transitional defect''' | ||
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* Shunting is minimal | * Shunting is minimal | ||
** Due to a small and restrictive ventricular septal defect. | ** Due to a small and restrictive ventricular septal defect. | ||
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* Similar to those of partial canal defect | |||
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Revision as of 08:46, 20 April 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]
Overview
Pathophysiology
Physiology
- AV canal connects the atria to the ventricles.[1]
- At four to five weeks of gestation, the superior and inferior endocardial cushions of the common AV canal fuse.[2]
- Results in the formation of the mitral and tricuspid valve and the AV septum.
Pathophysiology
Level of left-to-right shunting | Pathophysiology | |
---|---|---|
Complete defect | At the atrial and ventricular levels |
|
Partial defect | At the level of the primum atrial septal defect |
|
Transitional defect |
|
|
Genetics
- There is a strong association between AV canal defects and Down syndrome.[3]
- Chromosome 21 has been designated an AV canal critical region.
- Trisomy 21 have an AV canal defect, usually the complete form
Associated Conditions
Common cardiac conditions associated with endocardial cushion defect include:[4][5]
- Tetralogy of Fallot
- Transposition of the great arteries
- Patent ductus arteriosus
- Coarctation of the aorta
- Absent atrial septum
- Persistent left superior vena cava
- Anomalous pulmonary venous connection
References
- ↑ Wenink AC, Zevallos JC (January 1988). "Developmental aspects of atrioventricular septal defects". Int. J. Cardiol. 18 (1): 65–78. doi:10.1016/0167-5273(88)90031-9. PMID 3343065.
- ↑ VAN MIEROP LH, ALLEY RD, KAUSEL HW, STRANAHAN A (January 1962). "The anatomy and embryology of endocardial cushion defects". J. Thorac. Cardiovasc. Surg. 43: 71–83. PMID 13924605.
- ↑ Korenberg JR, Bradley C, Disteche CM (February 1992). "Down syndrome: molecular mapping of the congenital heart disease and duodenal stenosis". Am. J. Hum. Genet. 50 (2): 294–302. PMC 1682442. PMID 1531166.
- ↑ Peoples WM, Moller JH, Edwards JE (1983). "Polysplenia: a review of 146 cases". Pediatr Cardiol. 4 (2): 129–37. doi:10.1007/BF02076338. PMID 6878069.
- ↑ Karl TR (January 1997). "Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations". Semin. Thorac. Cardiovasc. Surg. 9 (1): 26–34. PMID 9109222.