Fourth Decade After Repair of Tetralogy of Fallot

This year marks the 60th year since the first successful intracardiac repair of tetralogy of Fallot (TOF).1 The repair, largely unchanged over decades, consists of ventricular septal defect closure and the relief of variable forms of right ventricular (RV) obstruction, usually with a transannular patch (TAP), and usually resulting in free pulmonary insufficiency (PI). Early in the experience with TOF repair, attention was on quantity of life—lifting the early dip in the survival curve. Palliative shunts were widely used to permit repair at a safer, older age. The dividends from a full relief of obstruction included excellent function for decades for patients formerly suffering from morbid or lethal disease. In this issue of Circulation , Cuypers and colleagues2 report findings of the third decennial follow-up of a cohort of early TOF repairs, the longest prospective study of this population to date. As such longitudinal evidence accrues, we are continually called on to reexamine both ends of the treatment timeline, although it is a wobbly yardstick that attempts to inform today’s best practices by measuring the sequelae of yesterday’s.Article see p 1944Early mortality after TOF repair is now very low, and the 10- to 20-year follow-up was encouraging. However, in the third postrepair decade, the Kaplan–Meier curve droops with progressive exercise intolerance, arrhythmia, right heart failure, and sudden death. Evidence points to ventricular scarring, chronic PI, RV hypertrophy, and declining left ventricular and RV performance as interrelated contributing factors. Some late risk may be modified at the front end, during …