8 year old female with history of Turner syndrome bicuspid aortic valve and coarctation repair in infancy. 1. PAPVR of left upper lobe to left innominate vein with resulting Qp:Qs of 1.37:1 and only mild dilation of right atrium and right ventricle.  2. Mild residual narrowing of second transverse segment at site of coarctation repair without hemodynamic significance. 3. Moderate post-stenotic dilation of proximal descending aorta which extends down to level of the diaphragm. 4. Bicuspid aortic valve with fusion of the right and left coronary cusps without stenosis or regurgitation.5. Normal LV mass.

 

Learning Points PAPVR frequently associated with Turner syndrome in addition to BAV and CoA.  Decision to repair is related to relative shunt and long term effect on pulmonary vasculature.  Qp:Qs of 1.37:1 falls within acceptable limits and is consistent with only mild RV enlargement.