Congenital cyanotic heart disease: Difference between revisions
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* Tetralogy of fallot | * Tetralogy of fallot | ||
**It is understood that tetralogy of fallot is the result of improper positioning of the outlet [[septum]].<ref name="pmid19094375">{{cite journal| author=Anderson RH, Jacobs ML| title=The anatomy of tetralogy of Fallot with pulmonary stenosis. | journal=Cardiol Young | year= 2008 | volume= 18 Suppl 3 | issue= | pages= 12-21 | pmid=19094375 | doi=10.1017/S1047951108003259 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19094375 }}</ref><ref name="pmid17420363">{{cite journal| author=Bashore TM| title=Adult congenital heart disease: right ventricular outflow tract lesions. | journal=Circulation | year= 2007 | volume= 115 | issue= 14 | pages= 1933-47 | pmid=17420363 | doi=10.1161/CIRCULATIONAHA.105.592345 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17420363 }}</ref><ref name="pmid19144126">{{cite journal| author=Bailliard F, Anderson RH| title=Tetralogy of Fallot. | journal=Orphanet J Rare Dis | year= 2009 | volume= 4 | issue= | pages= 2 | pmid=19144126 | doi=10.1186/1750-1172-4-2 | pmc=PMC2651859 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19144126 }}</ref> | |||
**In the normal [[heart]], the outlet septum is an indistinguishable component of the crista supraventricularis that communicates with the [[Septomarginal trabecula|septomarginal trabeculae]] to divide the [[Right ventricle|right]] and [[Left ventricle|left]] [[Ventricle|ventricular cavities]]. | |||
**In Tetralogy of Fallot, proper [[Ventricle (heart)|ventricular]] septation is perturbed by anterocephalad displacement of the outlet [[Septum (disambiguation)|septum]] relative to the [[septomarginal trabecula]]. | |||
**The direct consequence of this misalignment is an [[Overriding aorta|overriding aortic orifice]] and a [[ventricular septal defect]], resulting in an intracardiac [[Right-to-left shunt|right to left shunt]] of blood. | |||
**In addition, anterocephalad displacement of the outlet septum indirectly predisposes the [[pulmonary trunk]] to [[Pulmonary stenosis|stenosis]] in the setting of septoparietal trabecular [[hypertrophy]]. | |||
**Together, the displacement of the outlet [[septum]] coupled with the [[Hypertrophy (medical)|hypertrophic]] arrangement of the septoparietal trabeculae account for the three [[Anatomy|anatomical]] cardinal [[Defect|defects]] in Tetralogy of Fallot - [[Aorta|aortic]] dextroposition, [[Ventricular septal defect|interventricular communication]] ([[VSD]]), and [[pulmonary stenosis]]. | |||
**The fourth [[defect]] - [[right ventricular hypertrophy]] - is a [[hemodynamic]] consequence of these three [[Morphology|morphologic]] changes, as the [[right ventricle]] physiologically adapts to the increased [[resistance]] of a [[Pulmonary stenosis|stenotic pulmonary trunk]]. | |||
* Total anomalous pulmonary venous connection | * Total anomalous pulmonary venous connection | ||
**n this condition,the right side of heart is receiving blood both from pulmonary and systemic circulation.<ref name="pmid13378917">{{cite journal |vauthors=NEILL CA |title=Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return |journal=Pediatrics |volume=18 |issue=6 |pages=880–7 |date=December 1956 |pmid=13378917 |doi= |url=}}</ref><ref name="pmid13386206">{{cite journal |vauthors=CRAIG JM, DARLING RC, ROTHNEY WB |title=Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies |journal=Lab. Invest. |volume=6 |issue=1 |pages=44–64 |date=1957 |pmid=13386206 |doi= |url=}}</ref> | |||
**There is a mixing of oxygenated pulmonary venous blood with deoxygenated blood from systemic circulation. | |||
** The mixing of blood could occur at three levels i.e. supracardiac, infracardiac and cardiac. | |||
**In the former two the mixing occurs outside the heart and in latter inside the heart (right atrium) | |||
* Transposition of the great arteries | * Transposition of the great arteries | ||
* Truncus arteriosus | * Truncus arteriosus |
Revision as of 17:23, 14 April 2020
Congenital cyanotic heart disease Microchapters |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D. Eiman Ghaffarpasand, M.D. [2]
Overview
Congenital cyanotic heart disease is a group of heart defects that will causes neonatal cyanosis. The patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation. This can be caused by right-to-left or bidirectional shunting, or malposition of the great arteries. Such defects include persistent truncus arteriosus, total anomalous pulmonary venous connection, tetralogy of Fallot, transposition of the great vessels, and Ebstein's anomaly.
Classification
Congenital cyanotic heart disease may be classified according to anatomical defect into 5 subgroups:
- Tetralogy of fallot
- It is understood that tetralogy of fallot is the result of improper positioning of the outlet septum.[1][2][3]
- In the normal heart, the outlet septum is an indistinguishable component of the crista supraventricularis that communicates with the septomarginal trabeculae to divide the right and left ventricular cavities.
- In Tetralogy of Fallot, proper ventricular septation is perturbed by anterocephalad displacement of the outlet septum relative to the septomarginal trabecula.
- The direct consequence of this misalignment is an overriding aortic orifice and a ventricular septal defect, resulting in an intracardiac right to left shunt of blood.
- In addition, anterocephalad displacement of the outlet septum indirectly predisposes the pulmonary trunk to stenosis in the setting of septoparietal trabecular hypertrophy.
- Together, the displacement of the outlet septum coupled with the hypertrophic arrangement of the septoparietal trabeculae account for the three anatomical cardinal defects in Tetralogy of Fallot - aortic dextroposition, interventricular communication (VSD), and pulmonary stenosis.
- The fourth defect - right ventricular hypertrophy - is a hemodynamic consequence of these three morphologic changes, as the right ventricle physiologically adapts to the increased resistance of a stenotic pulmonary trunk.
- Total anomalous pulmonary venous connection
- n this condition,the right side of heart is receiving blood both from pulmonary and systemic circulation.[4][5]
- There is a mixing of oxygenated pulmonary venous blood with deoxygenated blood from systemic circulation.
- The mixing of blood could occur at three levels i.e. supracardiac, infracardiac and cardiac.
- In the former two the mixing occurs outside the heart and in latter inside the heart (right atrium)
- Transposition of the great arteries
- Truncus arteriosus
- Ebstein's anomaly
Differential diagnosis
Congenital cyanotic heart disease should be differentiated from other conditions that causes cyanosis:
References
- ↑ Anderson RH, Jacobs ML (2008). "The anatomy of tetralogy of Fallot with pulmonary stenosis". Cardiol Young. 18 Suppl 3: 12–21. doi:10.1017/S1047951108003259. PMID 19094375.
- ↑ Bashore TM (2007). "Adult congenital heart disease: right ventricular outflow tract lesions". Circulation. 115 (14): 1933–47. doi:10.1161/CIRCULATIONAHA.105.592345. PMID 17420363.
- ↑ Bailliard F, Anderson RH (2009). "Tetralogy of Fallot". Orphanet J Rare Dis. 4: 2. doi:10.1186/1750-1172-4-2. PMC 2651859. PMID 19144126.
- ↑ NEILL CA (December 1956). "Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return". Pediatrics. 18 (6): 880–7. PMID 13378917.
- ↑ CRAIG JM, DARLING RC, ROTHNEY WB (1957). "Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies". Lab. Invest. 6 (1): 44–64. PMID 13386206.