Tetralogy of Fallot (TOF) is a rare condition characterized by four cardiovascular defects at birth: Pulmonary valve stenosis, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. Although TOF is a surgical repairable condition, data on long-term survival following a repair are not available. This study aims to examine the long-term, transplant-free survival of TOF after surgical repair.
This retrospective cohort study included a total of 3,283 patients with TOF who survived the surgical repair procedure. Patient-associated and surgery-associated risk factors affecting survival were examined. The primary outcome of the study was transplant-free survival during the early and late phases after TOF surgical repair.
After 25 years of TOF repair, the survival rate was 94.5%. The multivariable analysis suggested an increased risk of early mortality in patients with staged repair (hazard ratio 2.68) and non-valve-sparing operation (HR 3.76). The presence of genetic abnormality was linearly associated with the risk of mortality in both early preoperative (HR 3.64) and late preoperative (HR 4.41) phase.
The research concluded that TOF surgical repair was associated with excellent survival. However, staged repair and non-valve-sparing operations were inversely associated with overall survival in the early post-repair phase. The risk of mortality was also higher in patients with a presence of genetic abnormality.