Sandbox:Pulmonary valve stenosis: Difference between revisions
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==Causes== | ==Causes== | ||
Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the pulmonary valve. The valve pathology can be congenital or acquired. The following is the list of causes: | Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the pulmonary valve. The valve pathology can be congenital or acquired. The following is the list of causes: | ||
*Congenital causes: Account for 95% of the cases and | *Congenital causes: Account for 95% of the cases and include: | ||
**Associated with congenital heart disease: | **Associated with congenital heart disease: | ||
***Tetralogy of Fallot | ***Tetralogy of Fallot | ||
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***Bicuspid pulmonary valve: Frequently associated with Tetralogy of Fallot. | ***Bicuspid pulmonary valve: Frequently associated with Tetralogy of Fallot. | ||
***Quadricuspid pulmonary valve: They are benign and an incidental finding. | ***Quadricuspid pulmonary valve: They are benign and an incidental finding. | ||
**Isolated pulmonic stenosis: | **Isolated pulmonic stenosis: The causes include as follows: | ||
***Acommissural pulmonary valves: Valves have a prominent systolic doming of the valve cusps and an eccentric orifice. | ***Acommissural pulmonary valves: Valves have a prominent systolic doming of the valve cusps and an eccentric orifice. | ||
***Dysplastic pulmonary valves: Thickened and deformed cusps with no commissural fusion. | ***Dysplastic pulmonary valves: Thickened and deformed cusps with no commissural fusion. | ||
Revision as of 22:10, 7 December 2016
Overview
Historical Perspective
Epidemiology and Demographics
Incidence of isolated PS is 1.5-6.5 per 10,000live births and accounts for 2 to 13% of all congenital heart lesions.
Causes
Pulmonary valve stenosis is due to a structural changes resulting from thickening and fusion of the pulmonary valve. The valve pathology can be congenital or acquired. The following is the list of causes:
- Congenital causes: Account for 95% of the cases and include:
- Associated with congenital heart disease:
- Tetralogy of Fallot
- Double outlet right ventricle
- Univentricular atrio-ventricular connection
- Atrioventricular canal defect
- Bicuspid pulmonary valve: Frequently associated with Tetralogy of Fallot.
- Quadricuspid pulmonary valve: They are benign and an incidental finding.
- Isolated pulmonic stenosis: The causes include as follows:
- Acommissural pulmonary valves: Valves have a prominent systolic doming of the valve cusps and an eccentric orifice.
- Dysplastic pulmonary valves: Thickened and deformed cusps with no commissural fusion.
- Other variations include of commissural malformation include: unicommissural pulmonary valve, bicuspid valve with fused commissures.
- Associated with congenital heart disease:
- Acquired Causes: These are less frequent and account for less than 5% of the cases.
- Carcinoid Syndrome: It is the most common acquired cause of PS.
- Post infectious causes
- Rheumatic heart disease
Pathophysiology
Anatomy
- Pulmonary valve is located at the distal part of the right ventricular outflow tract at the junction of the pulmonary artery.
- It is located anterior and superior to the aortic valve at the level of the third intercostal space and separated from the tricuspid valve by the infundibulum of the right ventricle.
- It is comprised of three equal sized, semilunar cusps or leaflets (right, left, anterior), nomenclature based on the corresponding aortic valve.
- The three cusps are joined by commissures and the cusps are thinner when compared to the aortic valve, due to a low pressure in the right ventricle.
- The area of the valve is related to body surface area and men usually have greater valve area when compared with women.[1]
- The normal orifice area is approximately around 3cm².[2]
- The pulmonary valve opens in the right ventricular systole allowing the deoxygenated blood to be delivered to the lungs.
- During the right ventricular diastole the pulmonary valves close completely to prevent regurgitation of blood into the right ventricle.
Pathogenesis
- Pulmonic valve stenosis can result from structural alterations resulting from congenital and acquired causes.
Genetics
Associated Conditions
History, Symptoms
Physical Examination
Diagnosis
Treatment
Guidelines
Medical Therapy
Surgical Therapy
Follow up
Prevention
Reflist</2> Template:WH Template:WS
- ↑ Capps SB, Elkins RC, Fronk DM (2000). "Body surface area as a predictor of aortic and pulmonary valve diameter". J Thorac Cardiovasc Surg. 119 (5): 975–82. doi:10.1016/S0022-5223(00)70092-4. PMID 10788818.
- ↑ Singh B, Mohan JC (1992). "Doppler echocardiographic determination of aortic and pulmonary valve orifice areas in normal adult subjects". Int J Cardiol. 37 (1): 73–8. PMID 1428292.