Alagille syndrome

Case contributed by Dr Jayanth Keshavamurthy

Presentation

Known patient with Alagille syndrome, branch pulmonary artery stenosis and a left pulmonary artery (LPA) stent.

Patient Data

Age: 6 years
Gender: Male
X-ray

Stable appearance of the median sternotomy wires with the most inferior wire fractured. Stable pulmonary artery vascular stent. 

No focal areas of consolidation or pleural effusions are identified.

The cardiac silhouette is not enlarged

Case Discussion

Surgery initially, thereafter multiple cardiac interventions for pulmonary artery stenosis with stents.

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