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In this condition, which has some similarities to Fallot's Tetralogy, there is a VSD with narrowing at the pulmonary valve. However the Pulmonary Valve leaflets (flaps) are rudimentary and non functional, resulting in severe Pulmonary Incompetence. The Pulmonary Arteries tend to be enlarged and may cause problems by compressing and narrowing air passages in the lungs, resulting in respiratory difficulty.
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