A 10 months old infant underwent repair of tetralogy of Fallot with hypoplastic annulus of the pulmonary valve, diminutive pulmonary arteries, right aortic arch and left superior vena cava. The right ventricular outflow tract was reconstructed using a monocusp patch. The postoperative course was unfavourably influenced by respiratory complications due to tracheal bronchus and hypoplasia of trachea, which were not diagnosed preoperatively. Extreme emphysema of the right upper andmiddle lobes compromised haemodynamics. Repeated reoperations were required. The upper and the middle lobes of the right lung had to be resected, the hypoplastic trachea reconstructed with a pericardial patch and pulmonary homograft inserted. One year later, homograft had to be replaced and tricuspid annuloplasty performed for pulmonary and tricuspid regurgitation and right ventricular dilatation as a consequence of increased pulmonary artery pressure. Three years after the original surgery the patient remains in good clinical condition.

        Key words: congenital heart disease, tetralogy of Fallot, hypoplastic trachea, tracheal bronchus, patch repair of trachea, heart surgery, complication, reoperation, child, infant.