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The pulmonary valve leaflets are thickened and have restricted opening. The right ventricle has extra work to do and becomes thicker. If the valve is very tight, blueness will present early in life. If the obstruction is moderate, a murmur will be picked up at routine examination.
Mild obstruction is tolerated very well over a long period of time. Moderate and severe obstruction requires relief. The valve can be stretched and this is usually performed by a balloon catheter. Generally, the results of ballooning these valves have been very good. In some cases the ballooning procedure is not possible, particularly in small babies where the obstruction is very severe, and in some older children whose valves are very thick. This may be associated with Noonan’s syndrome.
In these cases, an operation to stretch or remove the valve, would be required. The long term outlook is good. Mild leaking is well tolerated. Antibiotic prophylaxis requirement is being debated.