Severe Left and Right Pulmonary Artery Stenosis

Patient :
5 months old boy, 7 kg weight with syndrome of Williams-Beuren,
Severe Left and Right Pulmonary Artery Stenosis.

Findings :
Severe RV pressure load (systemic level) due to main, right and left pulmonary artery stenosis. Mild supravalvular aortic stenosis (gradient 10 mmHg), no aortic arch obstruction.

3DRA and stent-procedure :
Performing an initial double location 3DRA (Siemens DynaCT) angiography enabled us to get all anatomical information - right and left heart - in one run instead of 3 to four separate biplane angiographies (RV, PA bifurcation, LV and Ascendens). Virtual achievable angulations would not have been possible with conventional biplane systems. By duplicating the initial rotational angiography on the workstation two identical DynaCT datasets were obtained and merged into each other.

Herewith right and left heart anatomy could have been visualized separately with two different colours and the opportunity to blend between the two datasets. Bare metal stents (8x16 and 8x20mm, later expandable up to 16mm) were implanted in both RPA and LPA. 3DRA (single location injection into RV) and DynaCT reconstruction were performed to proof the absence of dissection. The 3D comparison between the pre and post status is visualized by merging the pre and post DynaCT dataset into each other."

Rotational Angiography in RPA LPA Hypoplasia before stentimplantation:

3DRA before and after RPA LPA stentimplantation: