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Tracheobronchial obstruction and unilateral hypoplasia of the pulmonary artery are serious barriers to successful Fontan completion. We describe a 5-year old girl with left bronchial obstruction, hypoplasia of the left pulmonary artery and a single ventricle (double inlet left ventricle). She had undergone external stenting to treat left bronchial stenosis at 3 years of age, bidirectional cavopulmonary anastomosis and Damus-Kaye-Stansel anastomosis at 4 years of age, intrapulmonary artery septation 1 year later and, finally, completion of the Fontan operation with excellent postoperative haemodynamics.

Fontan completion after external stenting of the left bronchus and intrapulmonary artery septation for left pulmonary artery hypoplasia