CTICasesWilliams Syndrome with Congenital Pulmonary Vein Stenosis
Imaging findings
Contrast-enhanced CT of the chest demonstrates status post surgical repair of supravalvular aortic stenosis (demonstrated by sternal wires). The right pulmonary veins are normal, but the left superior and left inferior pulmonary veins show severe, abrupt focal narrowing/stenosis at their insertion into the left atrium, with upstream dilation of the more distal pulmonary vein segments. No prior pulmonary vein ablation has been performed.
Key takeaways
Williams syndrome is a multisystem congenital disorder classically associated with supravalvular aortic stenosis and peripheral pulmonary artery stenosis. However, it can also rarely be associated with congenital pulmonary vein stenosis, which can present as progressive narrowing of the left atrium-pulmonary vein junction. This congenital etiology should be considered in pediatric or young adult patients with Williams syndrome who present with pulmonary venous congestion or unexplained pulmonary hypertension, in the absence of a history of atrial fibrillation ablation.
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