Tetralogy of Fallot

December 19, 2018

Long-term Outcomes of Tetralogy of FallotA Study From the Pediatric Cardiac Care Consortium

Clayton A. Smith, MD^1,2; Courtney McCracken, PhD^1,2; Amanda S. Thomas, MSPH¹; et alLogan G. Spector, PhD³; James D. St Louis, MD⁴; Matthew E. Oster, MD, MPH^1,2; James H. Moller, MD⁵; Lazaros Kochilas, MD, MSCR^1,2

Author Affiliations Article Information

JAMA Cardiol. Published online December 19, 2018. doi:10.1001/jamacardio.2018.4255

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Key Points

Question  What are the patient and surgical characteristics affecting long-term transplant-free survival following surgical tetralogy of Fallot repair?

Findings  In this cohort study of 3283 patients with tetralogy of Fallot, survival following complete repair was 98.6%, 97.8%, 97.1%, 95.5%, and 94.5% for 1-year, 5-year, 10-year, 20-year, and 25-year survival, respectively, with an early peaking hazard of mortality shortly after repair. Statistically significant associations with decreased long-term survival included staged repair, non–valve-sparing operation, repair in earlier surgical era, and presence of a genetic abnormality.

Meaning  Overall long-term transplant-free survival in repaired tetralogy of Fallot is excellent, with several factors affecting survival, some of which may be modifiable such as planning of the surgical strategy.