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Author Notes:

Lazaros Kochilas, MD, MSCR, Department of Pediatrics, Emory University School of Medicine, 2835 Brandywine Rd, Ste 300, Atlanta, GA 30341 (lazaros.kochilas@emory.edu)

Drs Kochilas, the senior author, and McCracken, the biostatistician performing the analysis, had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Concept and design: Smith, Spector, St Louis, Oster, Kochilas.

Acquisition, analysis, or interpretation of data: All authors.

Drafting of the manuscript: Smith, McCracken, Thomas, St Louis.

Critical revision of the manuscript for important intellectual content: Smith, McCracken, Spector, St Louis, Oster, Moller, Kochilas.

Statistical analysis: Smith, McCracken, Thomas, Spector, Oster.

Obtained funding: Spector, St Louis, Kochilas.

Administrative, technical, or material support: Thomas, Spector, Oster, Moller, Kochilas.

Study supervision: Oster, Kochilas.

Conflict of Interest Disclosures: None reported.

We thank the program directors and data collection coordinators from the participating PCCC centers; without their effort and dedication, this work could not have been completed. No compensation was received by any of the funding sponsors for the work done by the PCCC centers.

Subjects:

Research Funding:

Supported by the National Heart, Lung, and Blood Institute 5R01 HL122392-04 and the National Institutes of Health CTSA Award 8UL1TR000114-02.

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Cardiac & Cardiovascular Systems
  • Cardiovascular System & Cardiology
  • DUCTAL-DEPENDENT TETRALOGY
  • NATIONAL DEATH INDEX
  • FOLLOW-UP
  • POSTOPERATIVE OUTCOMES
  • SURGICAL-MANAGEMENT
  • REPAIRED TETRALOGY
  • RISK
  • MORTALITY
  • SURVIVAL

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

Tools:

Journal Title:

JAMA CARDIOLOGY

Volume:

Volume 4, Number 1

Publisher:

, Pages 34-41

Type of Work:

Article | Final Publisher PDF

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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© 2018 American Medical Association. All rights reserved.

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