Although dilatation of the pulmonary artery (PA) is characteristic of pulmonic valve stenosis (PVS), the occurrence of a PA aneurysm in association with PVS is very rare. The authors describe a patient with severe, dome-shaped PVS who developed a 6.5 cm pulmonary trunk aneurysm. Long-term follow-up after surgical valvotomy demonstrated that the size of the aneurysm remained unchanged. Pertinent literature and implications on the natural history and management of these aneurysms are discussed.
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