Tetralogy of Fallot (TOF) is the most common congenital cyanotic heart disease. Surgical repair of TOF was shown in 1954 to have a long-term survival rate of 77%, however, more recent estimates of long-term survival have been biased by selection, differences in treatment strategies, and variation in health care systems. As such, an updated evaluation of long-term survival following TOF repair is needed. In this multicenter retrospective cohort study, 3,283 patients in the Pediatric Cardiac Care Consortium (PCCC) who survived repair for simple TOF were followed up to assess the long-term transplant-free survival of TOF following surgery. Of the study population, 3,168 underwent primary repair, and 762 had a staged approach with initial shunt followed by repair. Researchers found that the long-term transplant-free survival after TOF repair was 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% at 1, 5, 10, 20, and 25 years after repair, respectively. Univariate analysis revealed that staged repair in the early postoperative phase (<6 years) was associated with increased late mortality as compared with primary repair (HR 3.45, 95% CI 2.02 to 5.89), and with non-valve-sparing repair in the early postoperative phase as compared with valve-sparing repair (HR 4.56, 95% CI 1.86 to 11.20). Presence of genetic abnormality was associated with a higher risk of premature mortality in the late postoperative phase (>6 years) (HR 4.41, 95% CI 2.62 to 7.44). Overall, this study showed a very high long-term survival rate after simple TOF repair, and that staged and non-valve sparing operations for TOF were associated with a lower long-term survival rate.
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