Severe hemoptysis in a child after the Fontan procedure

There is an increased incidence of pulmonary hemorrhage and hemoptysis among patients with congenital heart disease (CHD). The pathophysiology of pulmonary hemorrhage in CHD includes pulmonary hypertension, pulmonary venous congestion, aortopulmonary collaterals, pulmonary arteriovenous malformations, and dilated bronchial arteries. We present the case of a 6-year old boy who required treatment for massive hemoptysis after staged palliation for hypoplastic left heart syndrome (HLHS). Effective management of this life threatening entity is described as well as the anesthetic implications of performing rigid bronchoscopy in a patient with the Fontan circulation and massive hemoptysis.