Outcome after surgical repair of tetralogy of Fallot

Introduction: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Intracardiac correction was pioneered by Walton Lillehei in 1955 and has since then gone through major developments. The aim of this study was to provide a systematic literature review of published results on the long-term outcome of complete surgical correction of TOF.
Methods: MEDLINE, PubMed, Embase, Web of Science, Cochrane, and Google Scholar were systematically searched for literature published between January 2000 and July 2018. Pooled estimates with a random effects model after log-transformation were calculated for mortality and reintervention. Potential heterogeneity was assessed by subgroup analyses and meta-regression.
Results: A total of 143 papers of 137 distinct cohorts comprising 21,427 patients and total follow-up duration of 147,430 patient-years were included. Overall mean age at correction was 3.7 ± 5.6 years, but excluding papers exclusively focusing on correction in adults yielded a mean age of 0.5 ± 2.5 years at correction. Previous palliative shunts (107 studies), a transventricular approach (81 studies), and a transannular patch (124 studies) were used in 16% (range 0%-78%), 39% (range 0%-100%), and 49% (range 0%-100%) of the patients. respectively. In case a transannular patch was used, monocusp reconstruction was applied in 15% (range 0%-100%) (49 studies). The most common genetic abnormality was Down syndrome, with a pooled estimated prevalence of 4.6% (range 0%-12.3%). The pooled estimates of early and late mortality were 2.84% (95% confidence interval [CI], 2.34-3.45) and 0.42%/year (95% CI, 0.33-0.54), respectively. The pooled estimate of late cardiac mortality was 0.26%/year (95% CI, 0.21-0.34). Valve-related mortality and non–valve-related mortality had pooled estimates of 0.20%/year (95% CI, 0.15-0.26) and 0.17%/year (95% CI, 0.12-0.22), respectively. The pooled estimate of reintervention was 2.26%/year (95% CI, 1.86-2.75).
Conclusions: TOF can be surgically corrected at a young age with low perioperative and long-term mortality. Life-long intensive follow-up and substantial reintervention rates characterize the clinical course.