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Original Investigation
January 2019

Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium

Author Affiliations
  • 1Department of Pediatrics, Emory University School of Medicine
  • 2Children’s Healthcare of Atlanta, Atlanta, Georgia
  • 3Department of Pediatrics, University of Minnesota, Minneapolis
  • 4Department of Pediatric Surgery, University of Missouri, Kansas City School of Medicine
  • 5Department of Internal Medicine, University of Minnesota, Minneapolis
JAMA Cardiol. 2019;4(1):34-41. doi:10.1001/jamacardio.2018.4255
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.

Abstract

Importance  Tetralogy of Fallot (TOF) is a surgically repairable form of cyanotic congenital heart disease. Multicenter data for long-term survival following repair are sparse.

Objective  To evaluate the long-term transplant-free survival of TOF by surgical strategy adjusted for era and patient characteristics.

Design, Setting, and Participants  Retrospective cohort study enriched with data from the National Death Index and the Organ Procurement and Transplantation Network through 2014. Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for interventions for congenital heart disease. The cohort included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were enrolled in the PCCC registry between 1982 and 2003 and survived surgical repair of simple TOF. Data were analyzed between September 2015 and April 2018.

Exposures  We examined patient-associated and surgery-associated risk factors affecting survival.

Main Outcomes and Measures  We analyzed the transplant-free survival during early (<6 years) and late (≥6 years) phase after TOF surgical repair.

Results  Of the 3283 patients who survived repair for simple TOF and met the study’s inclusion criteria, 56.4% were male and 43.6% were female. Twenty-five–year survival following TOF repair was 94.5%. Multivariable analysis demonstrated increased risk of early mortality with staged repair (HR, 2.68; 95% CI, 1.59-4.49) and non–valve-sparing operation (HR, 3.76; 95% CI, 1.53-9.19). Presence of a genetic abnormality was associated with increased risk of death both in the early (HR, 3.64; 95% CI, 2.05-6.47) and late postoperative phase (HR, 4.41; 95% CI, 2.62-7.44).

Conclusions and Relevance  Long-term survival after simple TOF repair is excellent. Staged repair and non–valve-sparing operations were negatively associated with survival in the early postrepair phase but not the late postrepair phase. These data are important for patients with repaired TOF and their caretakers and may guide surgical strategies for optimizing the long-term outcomes of this population.

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