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

• Type of Material: Article
• Authors: Clayton Smith, Emory University; Courtney McCracken, Emory University; Amanda S. Thomas, Emory University; Logan G. Spector, University of Minnesota; James D. St Louis, University of Missouri; Matthew Oster, Emory University; James H. Moller, University of Minnesota; Lazaros Kochilas, Emory University
• Language: English
• Date: 2019-01-01
• Publisher: AMER MEDICAL ASSOC
• Publication Version: Final Published Version
• Copyright Statement: © 2018 American Medical Association. All rights reserved.
• Final Published Version (URL): http://dx.doi.org/10.1001/jamacardio.2018.4255
• Title of Journal or Parent Work: JAMA CARDIOLOGY
• Volume: 4
• Issue: 1
• Start Page: 34
• End Page: 41
• Grant/Funding Information: Supported by the National Heart, Lung, and Blood Institute 5R01 HL122392-04 and the National Institutes of Health CTSA Award 8UL1TR000114-02.
• Keywords: DUCTAL-DEPENDENT TETRALOGY; FOLLOW-UP; REPAIRED TETRALOGY; RISK; NATIONAL DEATH INDEX; MORTALITY; Cardiovascular System & Cardiology; Life Sciences & Biomedicine; SURVIVAL; Cardiac & Cardiovascular Systems; Science & Technology; SURGICAL-MANAGEMENT; POSTOPERATIVE OUTCOMES
• Research Categories: Biology, Anatomy; Health Sciences, Health Care Management; Health Sciences, Medicine and Surgery

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.