Long-term survival after repair of tetralogy of Fallot.

Abstract

Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease. Long-term survival has improved because of lower operative mortality and surgical repair in infancy or early childhood prior to the development of cardiac compromise from chronic hypoxia. Thus, more people with repaired TOF will survive to adulthood and will be interested in insurance coverage. This paper reported 36-year follow-up of 490 patients observed for a mean duration of 25 years after surgical repair of TOF; 42 deaths occurred, 62% of which were due to cardiac causes, principally sudden cardiac death and congestive cardiac failure. Mortality ratios were generally highest for the first 10 years of follow-up, ranging from 309% (ages < 10 years) to 714% (ages 20-39 years). By age, mortality ratios were stable and relatively similar for ages to 19 years and variable at older ages due to the small exposure. Most patients were actively at work (70.8%), homemakers (20.1%), or retired (6.1%); 3% were unable to work. For patients < 10 years of age and 10-19 years of age at the time of surgery who were alive after 1 year after the repair of the TOF, excess mortality persisted at least 30 years after surgery and did not vary a great deal with duration of the follow-up. Based on limited data, excess mortality for age group 20-39 years was higher compared with those in a younger age group during the first 10 years after surgery and decreased thereafter. Survival rates similar to those of the general population were attained in subjects who did not require treatment with a right ventricular outflow patch and who had normal hematocrit levels prior to surgery. Elevated morbidity risk was manifest by cardiac symptoms, arrhythmias, subsequent heart surgery, and disability in 3% of patients.