Smith CA, et al. - In this cohort study, researchers assessed the long-term transplant-free survival of tetralogy of Fallot (TOF), the most common form of cyanotic heart disease, by surgical strategy adjusted for era and patient characteristics. After simple TOF repair, long-term transplant-free survival is excellent. Findings suggested a negative association of staged repair and non–valve-sparing operations with survival in the early postrepair phase but not the late postrepair phase. These findings are significant for TOF repaired patients and their caretakers and can guide surgical strategies to optimize the long-term results of this population.

Methods

• Retrospective cohort study enriched through 2014 with data from the National Death Index and the Organ Procurement and Transplantation Network.
• Multicenter cohort from the Pediatric Cardiac Care Consortium (PCCC), a large, US-based clinical registry for congenital heart disease interventions.
• In this cohort, researchers included patients with adequate identifiers for linkage with the National Death Index and the Organ Procurement and Transplantation Network who were registered in the PCCC between 1982 and 2003 and survived surgical repair of simple TOF.
• Between September 2015 and April 2018, data were analyzed.
• Patient-associated and surgery-associated risk factors affecting survival were examined.
• After TOF surgical repair, the transplant-free survival during early (<6 years) and late (≥6 years) phase was analyzed.

Results

• As per data, of the 3283 patients who survived repair for simple TOF and met the inclusion criteria of the study, 56.4% were male and 43.6% were female.
• They observed that twenty-five–year survival following TOF repair was 94.5%.
• Multivariable analysis showed 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).
• 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), presence of a genetic abnormality was associated with increased risk of death.