Pulmonary valve stenosis
|Pulmonary valve stenosis|
|Other names: Valvular pulmonary stenosis, pulmonic stenosis|
|Symptoms||Few symptoms initially, may develop syncope, chest pain, and shortness of breath|
|Causes||Congenital heart disease, carcinoid syndrome|
|Diagnostic method||Suspected based on a systolic murmur, confirmed by ultrasound of the heart|
|Treatment||Balloon valvuloplasty, valve replacement|
|Frequency||0.4% at birth|
Pulmonary valve stenosis (PVS) is a type of heart valve disease in which there is narrowing of the pulmonary valve opening. Affected children often have no symptoms. When symptoms develop these may include syncope, chest pain, or shortness of breath.
The most common cause is congenital heart disease such as tetralogy of Fallot and Noonan syndrome. Other causes include carcinoid syndrome. Diagnosis may be suspected based on a systolic murmur and confirmed by ultrasound of the heart.
Treatment may may include balloon valvuloplasty and valve replacement. Outcomes are generally good. At birth it affected about 4 per 1,000 people. Initial descriptions of the condition at birth date from 1761 by John Baptist Morgagni. Surgical repair was first carried out in 1953.
Signs and symptoms
Among some of the symptoms consistent with pulmonary valve stenosis are the following:
In regards to the cause of pulmonary valve stenosis a very high percentage are congenital, the right ventricular flow is hindered (or obstructed by this). The cause in turn is divided into: valvular, external and intrinsic (when it is acquired).
Both stenosis of the pulmonary artery and pulmonary valve stenosis are forms of pulmonic stenosis (nonvalvular and valvular, respectively) but pulmonary valve stenosis accounts for 80% of pulmonic stenosis.
The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from another), which can cause high pulmonary pressure, and pulmonary hypertension. This however, does not mean the cause is always congenital.
The diagnosis of pulmonary valve stenosis can be achieved via echocardiogram, as well as a variety of other means among them are: ultrasound, in which images of the heart chambers in utero where the tricuspid valve has thickening (or due to Fallot's tetralogy, Noonan's syndrome, and other congenital defects) and in infancy auscultation of the heart can reveal identification of a murmur.
Some other conditions to contemplate (in diagnosis of pulmonic valvular stenosis) are the following:
In terms of treatment for pulmonary valve stenosis, valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case. Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used.
The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population.
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