Pulmonary artery aneurysm associated with a nonstenotic bicuspid pulmonic valve: A role for genetics?

Bicuspid pulmonic valves are quite uncommon, being described in only 0.1% of donor's hearts, while pulmonary artery aneurysms (PAAs) are even rarer, having been found in 8 out of 109,571 autopsies. This rarity makes it difficult to characterize the relationship between them.

We describe the case of a 66-year-old female who was found to have a bicuspid pulmonic valve and PAA (5.1 cm) on imaging by her cardiologist.

This case raises the question of whether the association between bicuspid semilunar valve disease and vascular wall anomalies is more genetic or hemodynamic. Even on the aortic side, despite the robust association between bicuspid aortic valves and thoracic aortic aneurysms, the mechanism still remains unclear. In our patient there was no significant gradient across the bicuspid pulmonic valve, suggesting that hemodynamics are not the primum mobile of this association.