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Without treatment, it is a highly life-threatening condition, so prognosis is poor. [35] [34] If surgery isn't performed in severe cases, the child can (and will) die, since the phenotype of pulmonary atresia is not compatible with life due to the pulmonary valve atresia resulting in reduced blood oxygenation. [9] [62] [63]
Pulmonary valve stenosis (PVS) is a heart valve disorder. Blood going from the heart to the lungs goes through the pulmonary valve, whose purpose is to prevent blood from flowing back to the heart. In pulmonary valve stenosis this opening is too narrow, leading to a reduction of flow of blood to the lungs. [1] [5]
When the right side of the heart is more underdeveloped than the left side, this is known as hypoplastic right heart syndrome. HRHS is known for the pulmonary valve, the tricuspid valve, right ventricle, and the pulmonary artery all failing to form properly. HRHS also causes the right ventricle to be a fair amount smaller than the left side.
Transcatheter pulmonary valve replacement (TPVR), also known as percutaneous pulmonary valve implantation (PPVI), is the replacement of the pulmonary valve via catheterization through a vein. It is a significantly less invasive procedure in comparison to open heart surgery and is commonly used to treat conditions such as pulmonary atresia .
Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. [2] The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal ...
Mechanical valve has greater durability, but requires anti-coagulation to reduce the risk of thrombosis. [3] Treatment of pulmonary hypertension will depend on the specific cause of the pulmonary hypertension. On top of this, the following may also be considered: diuretic, oxygen and anti-coagulant therapy. [3]
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