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Long-term outcomes of Tetralogy of Fallot: A study from the Pediatric Cardiac Care Consortium

JAMA Cardiology Jan 21, 2019

Smith CA, et al. - In this cohort study of 3,283 patients with tetralogy of fallot (TOF), researchers assessed the long-term transplant-free survival by surgical strategy adjusted for era and patient characteristics. The participants have survived surgical repair of simple TOF, with 56.4% being male and 43.6% female. 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 Pediatric Cardiac Care Consortium (PCCC) registry between 1982 and 2003. Data analysis was performed between September 2015 and April 2018. After TOF repair, the 25–year survival was 94.5%, indicating excellent long-term survival after simple TOF repair. Negative associations with survival were reported for staged repair and non–valve-sparing operations, these links were evident in the early postrepair phase (<6 years) but not the late postrepair phase (≥6 years). Both in the early and late postoperative phase, an increased risk of death was observed in association with the presence of a genetic abnormality.

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