KCNT1 mutations are associated with childhood epilepsy, developmental delay, and vascular malformations. We report a child with a likely pathogenic KCNT1 mutation (c.1885A>C, p.Lys629Glu) with recurrent pulmonary haemorrhage due to aortopulmonary collaterals successfully managed with coil embolisation followed by right upper lobectomy.

A significant contributing factor to the progression of late cyanosis in individuals undergoing Kawashima operation is pulmonary arteriovenous malformations. Following the Fontan procedure, arteriovenous malformations may regress. However, in cases with extensive malformations causing severe cyanosis, lobectomy can also be a possible treatment approach. Thereby, we present our two-step treatment strategy in a late Fontan completion complicated by arteriovenous malformations in a Kawashima patient.

Congenital lobar emphysema is often associated with CHD in early infancy; however, the surgical strategy for this condition is still controversial. We report three successful cases of emphysematous lung lobectomy before the surgical repair of associated CHD. Aggressive lobectomy preceding cardiac interventions is advised when the management of congenital lobar emphysema is difficult.