PMC4521322_01
A 6 year old girl presented with these classical findings of peripheral pulmonary stenosis (PPS) and supravalvar aortic stenosis (SVAS) : A) Depiction of the SVAS upon injection into the coronary sinuses. B-C) Visualization of the diffuse bilateral PPS on pulmonary angiography.
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F1
Cardiac catheterization confirmed the diagnosis of supravalvar aortic stenosis with trivial aortic regurgitation (Figure 1A). Right ventricular pressures were nearly systemic (114/14 vs. 126/60 mmHg) and angiography revealed a diffuse, extensive narrowing of both branch pulmonary arteries (Figure 1B-C).
A-C) CT-angiographic findings in the same patient in different angulations delineating the diffuse aspect of the PPS, but also some osteal stenosis at the LPA and further distal in both branch pulmonary arteries (arrows). D) 3D-reconstruction of the CT-angiographic dataset.
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F2
Here, the main pulmonary artery (6.5x7.0mm), right (5.0mm) and left branch pulmonary arteries (5.3 mm) were estimated to be of reduced size (Figure 2).