Cyanotic heart defect pathophysiology: Difference between revisions
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===Total Anomalous Pulmonary Venous Connection=== | ===Total Anomalous Pulmonary Venous Connection=== | ||
In this condition,the right side of heart is receiving blood both from pulmonary and systemic circulation. 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) | |||
* In this condition,the right side of heart is receiving blood both from pulmonary and systemic circulation. | |||
* 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) | |||
===Hypoplastic Left Heart Syndrome=== | ===Hypoplastic Left Heart Syndrome=== | ||
In patients with hypoplastic left heart syndrome, the left side of the heart is unable to send enough blood to the body. As a result, the right side of the heart must maintain the circulation for both the lungs and the body. The right ventricle can support the circulation to both the lungs and the body for a while, but this extra workload eventually causes the right side of the heart to fail. | * In patients with hypoplastic left heart syndrome, the left side of the heart is unable to send enough blood to the body. | ||
* As a result, the right side of the heart must maintain the circulation for both the lungs and the body. | |||
* The right ventricle can support the circulation to both the lungs and the body for a while, but this extra workload eventually causes the right side of the heart to fail. | |||
===Transpostion of Great Arteries=== | ===Transpostion of Great Arteries=== | ||
The pulmonary and systemic circulations function in parallel, rather than in series. Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed and deoxygenated systemic venous blood returns into right atrium and ventricle which is subsequently pumped back into systemic circulation.<ref name="pmid17159076">{{cite journal |vauthors=Warnes CA |title=Transposition of the great arteries |journal=Circulation |volume=114 |issue=24 |pages=2699–709 |date=December 2006 |pmid=17159076 |doi=10.1161/CIRCULATIONAHA.105.592352 |url=}}</ref> | |||
* The pulmonary and systemic circulations function in parallel, rather than in series. | |||
* Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed and deoxygenated systemic venous blood returns into right atrium and ventricle which is subsequently pumped back into systemic circulation.<ref name="pmid17159076">{{cite journal |vauthors=Warnes CA |title=Transposition of the great arteries |journal=Circulation |volume=114 |issue=24 |pages=2699–709 |date=December 2006 |pmid=17159076 |doi=10.1161/CIRCULATIONAHA.105.592352 |url=}}</ref> | |||
===Truncus Arteriosus=== | ===Truncus Arteriosus=== | ||
In truncus arteriosus, the pulmonary arteries are connected to the aorta. A decrease in PVR at birth causes a left to right shunt with evidence of congestive heart failure. These patients have a very high incidence of pulmonary hypertension and vascular disease. | |||
* In truncus arteriosus, the pulmonary arteries are connected to the aorta. | |||
* A decrease in PVR at birth causes a left to right shunt with evidence of congestive heart failure. | |||
* These patients have a very high incidence of pulmonary hypertension and vascular disease. | |||
===Tricuspid Atresia=== | ===Tricuspid Atresia=== | ||
In tricuspid atresia, there is no continuity between the right atrium and right ventricle. Blood from superior vena cava and inferior vena cava is forced across intra atrial connection into the left heart. As a consequence, oxygen saturation in the left atrial blood is diminished. | |||
* In tricuspid atresia, there is no continuity between the right atrium and right ventricle. Blood from superior vena cava and inferior vena cava is forced across intra atrial connection into the left heart. | |||
* As a consequence, oxygen saturation in the left atrial blood is diminished. | |||
==Genetics== | ==Genetics== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-In-Chief: Keri Shafer, M.D. [2], Atif Mohammad, M.D.; Kalsang Dolma, M.B.B.S.[3]
Overview
Pathophysiology
Tetrology 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
- In this condition,the right side of heart is receiving blood both from pulmonary and systemic circulation.
- 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)
Hypoplastic Left Heart Syndrome
- In patients with hypoplastic left heart syndrome, the left side of the heart is unable to send enough blood to the body.
- As a result, the right side of the heart must maintain the circulation for both the lungs and the body.
- The right ventricle can support the circulation to both the lungs and the body for a while, but this extra workload eventually causes the right side of the heart to fail.
Transpostion of Great Arteries
- The pulmonary and systemic circulations function in parallel, rather than in series.
- Oxygenated pulmonary venous blood returns to the left atrium and left ventricle but is recirculated to the pulmonary vascular bed and deoxygenated systemic venous blood returns into right atrium and ventricle which is subsequently pumped back into systemic circulation.[4]
Truncus Arteriosus
- In truncus arteriosus, the pulmonary arteries are connected to the aorta.
- A decrease in PVR at birth causes a left to right shunt with evidence of congestive heart failure.
- These patients have a very high incidence of pulmonary hypertension and vascular disease.
Tricuspid Atresia
- In tricuspid atresia, there is no continuity between the right atrium and right ventricle. Blood from superior vena cava and inferior vena cava is forced across intra atrial connection into the left heart.
- As a consequence, oxygen saturation in the left atrial blood is diminished.
Genetics
[Disease name] is transmitted in [mode of genetic transmission] pattern.
OR
Genes involved in the pathogenesis of [disease name] include:
- [Gene1]
- [Gene2]
- [Gene3]
OR
The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
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.
- ↑ Warnes CA (December 2006). "Transposition of the great arteries". Circulation. 114 (24): 2699–709. doi:10.1161/CIRCULATIONAHA.105.592352. PMID 17159076.