The 25-year survival rate for patients undergoing surgical repair for Tetralogy of Fallot (TOF), a form of cyanotic congenital heart disease, is 94.5%, with staged repairs and non-valve-sparing operations showing increased risk of early mortality, and genetic abnormalities increasing risk at ≥6 years post-surgery, according to a study published in JAMA Cardiology.

For this retrospective cohort study, researchers assessed the long-term survival rates of patients with TOF after non-transplant surgical repair by using registry data from 1982–2014 from the Pediatric Cardiac Care Consortium, the Organ Procurement and Transplantation Network, and the National Death Index.

Primary study outcomes were survival rates for <6 years (early) and ≥6 years (late) post-surgical repair for TOF, and the surgery and patient-associated risk factors that affected survival.

The 1-year survival rate following TOF repair in the cohort (N=3283, 43.6% women and 56.4% men) was 98.6%, decreasing to 97.8% at 5 years; 97.1% at 10 years; 95.5% at 20 years; and 94.5% at 25 years, with an early steep mortality hazard increase peaking shortly after repair, followed by a late, gradual hazard increase.

Increased mortality risks were seen in the early post-surgical phase with non-valve-sparing operations and staged repair (hazard ratio [HR], 3.76; 95% CI, 1.53–9.91 and HR, 2.68; 95% CI, 1.59–4.49, respectively).

Genetic abnormalities were associated with increased mortality risk in both early and late post-surgical phases (HR, 3.64; 95% CI, 2.05–6.47 and HR, 4.41; 95% CI, 2.62–7.44, respectively).

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Study investigators conclude, “Long-term transplant-free survival beyond the first 6 years… [is] influenced primarily by the coexistence of an associated genetic condition. Within the 25-year follow-up period, most deaths were associated with the underlying diagnosis of TOF and mediated by arrhythmias and congestive heart failure.”

Reference

Smith CA, McCracken C, Thomas AS, et al. Long-term outcomes of Tetralogy of Fallot: A study from the Pediatric Cardiac Care Consortium [published online December 19, 2018]. JAMA Cardiol. doi: 10.1001/jamacardio.2018.4255