Infective endocarditis is one of the complications encountered in patients with uncorrected tetralogy of Fallot; however, there have been only limited reports on surgical treatment of this condition. A 38-year-old man with uncorrected tetralogy of Fallot with pulmonary atresia previously palliated with modified Blalock-Taussig shunt was diagnosed with aortic valve infective endocarditis and developed severe aortic regurgitation. He underwent urgent aortic valve replacement, during which intermittent hypothermic circulatory arrest was necessary to achieve adequate myocardial protection and bloodless surgical field due to massive arterial return into the left ventricle from the collateral pulmonary circulation.
Keywords: Adult congenital heart disease; Aortic valve replacement; Aortopulmonary collateral arteries; Infective endocarditis; Tetralogy of Fallot with pulmonary atresia.