Uncorrected tetralogy of Fallot: adult presentation in the 61st year of life

Abstract

Tetralogy of Fallot (TOF) is the commonest form of cyanotic congenital heart defect after infancy [Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults-Second of Two Parts. NEJM 2000; 342(5):334-342.]. There are few studies assessing the risk of surgical correction in adult patients and long-term survival into the fourth decade of life is rare. The case history is described of a 61-year old female presenting with probable viral myocarditis. Subsequent investigations revealed an underlying diagnosis of tetralogy of Fallot. The patient remains asymptomatic despite persistent hypoxia. Potential factors contributing to longevity in this case are relatively good pulmonary blood flow via large branch pulmonary arteries, and the possible gradual development of right ventricular outflow tract obstruction over a long time period.