Bilateral branch pulmonary arterial reconstruction in the Alagille syndrome: The technique and its pitfalls

Abstract

This tutorial describes in detail the surgical technique of bilateral branch pulmonary arterial reconstruction in a 10-month-old boy with Alagille syndrome and advanced liver disease. The procedure was performed via a standard median sternotomy and moderate hypothermia and involves bilateral pulmonary branch arterioplasties combined with relief of valvular and supravalvular pulmonary stenosis and subtotal closure of secundum atrial septal defect. The patient presented with systemic/suprasystemic right ventricular pressure.

Keywords: Homograft; Pulmonary artery; Pulmonary artery surgery; Right branch of the pulmonary artery; Supravalvular stenosis.