Persistent truncus arteriosus pathophysiology & etiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Diagrams to illustrate the transformation of the [[bulbus cordis]]. Ao. [[Truncus arteriosus]]. Au. [[Atrium (heart)|Atrium]]. B. [[Bulbus cordis]]. RV. [[Right ventricle]]. LV. [[Left ventricle]]. P. [[Pulmonary artery]]. | Diagrams to illustrate the transformation of the [[bulbus cordis]]. Ao. [[Truncus arteriosus]]. Au. [[Atrium (heart)|Atrium]]. B. [[Bulbus cordis]]. RV. [[Right ventricle]]. LV. [[Left ventricle]]. P. [[Pulmonary artery]].<ref name="pmid3207487">{{cite journal |vauthors=Fuglestad SJ, Puga FJ, Danielson GK, Edwards WD |title=Surgical pathology of the truncal valve: a study of 12 cases |journal=Am J Cardiovasc Pathol |volume=2 |issue=1 |pages=39–47 |date=1988 |pmid=3207487 |doi= |url=}}</ref><ref name="pmid3797293">{{cite journal |vauthors=Butto F, Lucas RV, Edwards JE |title=Persistent truncus arteriosus: pathologic anatomy in 54 cases |journal=Pediatr Cardiol |volume=7 |issue=2 |pages=95–101 |date=1986 |pmid=3797293 |doi=10.1007/BF02328958 |url=}}</ref> | ||
[[Image:Gray469.png]] | [[Image:Gray469.png]] | ||
In normal circulation, the pulmonary artery comes out of the right ventricle and the aorta comes out of the left ventricle, which are separate from each other. Coronary arteries (which supply blood to the heart muscle) come out of the aorta just above the valve at the entrance of the aorta. | |||
In truncus arteriosus, a single artery comes out of the ventricles. There is usually also a large hole between the two ventricles (ventricular septal defect). As a result, the blue (without oxygen) and red (oxygen-rich) blood mix. | |||
Some of this mixed blood goes to the lungs, some goes to the coronary arteries, and the rest goes to the body. Usually, too much blood is sent to the lungs. | |||
If this condition is not treated, two problems occur: | |||
* Too much blood circulation in the lungs may cause extra fluid to build up in and around them, making it difficult to breathe. | |||
* The blood vessels to the lungs become permanently damaged. Over time, it becomes very hard for the heart to force blood to them. This is called pulmonary hypertension and it can be life-threatening. | |||
===Gross Pathology=== | |||
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<gallery heights="175" widths="175"> | |||
Image:Congenital heart defect 0035.jpg|Truncus Arteriosus with Subvalvular Ventricular Septal Defect: Gross, natural color, an excellent view of subvalvular defect. Quadricuspid truncus valve and type I origin of pulmonary arteries | |||
Image:Congenital heart defect 0036.jpg|Truncus Arteriosus with Subvalvular Interventricular Septal Defect: Gross, natural color, defect is shown from the right side (view toward right ventricular outlet) | |||
Image:Congenital heart defect 0037.jpg|Truncus Arteriosus with Subvalvular Interventricular Septal Defect: Gross natural color excellent view of lesion looking at opened aortic ring with quadricuspid aortic valve. A large subvalvular defect (origin of pulmonary arteries is at forceps) | |||
</gallery> | |||
</div> | |||
===Associated Conditions=== | |||
Anatomical changes associated with this disorder includes: | |||
* single artery arising from the two [[ventricle (heart)|ventricles]] which gives rise to both the aortic and pulmonary vessels | |||
* abnormal [[truncal valve]] | |||
* right sided [[aortic arch]] in about 30% of cases (not shown) | |||
* large [[ventricular septal defect]] | |||
* [[pulmonary hypertension]] | |||
* complete mixing occurring at level of the [[great vessel]] | |||
* right-to-left shunting of blood | |||
==References== | ==References== | ||
Latest revision as of 18:37, 4 May 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor-in-Chief: Keri Shafer, M.D. [2]
Overview
Pathophysiology
Diagrams to illustrate the transformation of the bulbus cordis. Ao. Truncus arteriosus. Au. Atrium. B. Bulbus cordis. RV. Right ventricle. LV. Left ventricle. P. Pulmonary artery.[1][2]
In normal circulation, the pulmonary artery comes out of the right ventricle and the aorta comes out of the left ventricle, which are separate from each other. Coronary arteries (which supply blood to the heart muscle) come out of the aorta just above the valve at the entrance of the aorta.
In truncus arteriosus, a single artery comes out of the ventricles. There is usually also a large hole between the two ventricles (ventricular septal defect). As a result, the blue (without oxygen) and red (oxygen-rich) blood mix.
Some of this mixed blood goes to the lungs, some goes to the coronary arteries, and the rest goes to the body. Usually, too much blood is sent to the lungs.
If this condition is not treated, two problems occur:
- Too much blood circulation in the lungs may cause extra fluid to build up in and around them, making it difficult to breathe.
- The blood vessels to the lungs become permanently damaged. Over time, it becomes very hard for the heart to force blood to them. This is called pulmonary hypertension and it can be life-threatening.
Gross Pathology
-
Truncus Arteriosus with Subvalvular Ventricular Septal Defect: Gross, natural color, an excellent view of subvalvular defect. Quadricuspid truncus valve and type I origin of pulmonary arteries
-
Truncus Arteriosus with Subvalvular Interventricular Septal Defect: Gross, natural color, defect is shown from the right side (view toward right ventricular outlet)
-
Truncus Arteriosus with Subvalvular Interventricular Septal Defect: Gross natural color excellent view of lesion looking at opened aortic ring with quadricuspid aortic valve. A large subvalvular defect (origin of pulmonary arteries is at forceps)
Associated Conditions
Anatomical changes associated with this disorder includes:
- single artery arising from the two ventricles which gives rise to both the aortic and pulmonary vessels
- abnormal truncal valve
- right sided aortic arch in about 30% of cases (not shown)
- large ventricular septal defect
- pulmonary hypertension
- complete mixing occurring at level of the great vessel
- right-to-left shunting of blood
References
- ↑ Fuglestad SJ, Puga FJ, Danielson GK, Edwards WD (1988). "Surgical pathology of the truncal valve: a study of 12 cases". Am J Cardiovasc Pathol. 2 (1): 39–47. PMID 3207487.
- ↑ Butto F, Lucas RV, Edwards JE (1986). "Persistent truncus arteriosus: pathologic anatomy in 54 cases". Pediatr Cardiol. 7 (2): 95–101. doi:10.1007/BF02328958. PMID 3797293.