Absent Pulmonary Valve Syndrome - Parent Info

  • This is a similar combination of problems to that seen in Fallot's Tetralogy.  A VSD with 'overriding' of the Aorta coincides with a narrow pulmonary valve.

    The difference is that the pulmonary valve flaps (leaflets) are very rudimentary (poorly formed). As a result the valve is severely incompetent as well as being narrow (stenotic). The pulmonary arteries are characteristically enlarged (rather than being small, as is the case with Fallot's Tetralogy). The large pulmonary arteries may put pressure on the adjacent air tubes (bronchi) within the lungs, leading to breathing problems. Affected babies sometimes have severe respiratory difficulties in early infancy and may need early surgery to repair the heart problems and to reduce the size of the enlarged pulmonary arteries. Surgical repair is essentially similar to that of Fallot's Tetralogy.

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