OBJECTIVE(S):
The natural history of standard (large, transmural right ventriculotomy) repair of tetralogy of Fallot (TOF) is associated with a concerning incidence of RV failure and re-operation. We believe that preserving the infundibulum using a TOF repair method with a mini-(< 5mm) or no ventricular incision optimizes RV function and confers long-term benefit. Over the past 13 years, we have uniformly applied this RV infundibulum sparing (RVIS) strategy. (Figure-1)
METHODS:
304 TOF patients underwent the RVIS strategy (7/1995-6/2008). Median weight and age at repair: 8kgs(3-62kgs) and 9months[2days-23years]. 17%(51) of patients required a systemic-to-pulmonary shunt.
RESULTS:
99% of patients had a mini-73%(222) or no 26%(79) ventricular incision. Post-operative morbidity included arrhythmias 4%(13), post-operative bleeding 2%(6), renal failure 1%(4), and neurological injury <1%(2). 30-day survival was 99.7%. Overall 1 and 7-year Kaplan-Meier survivals were 97% and 96%. 3.2%(10) of patients had re-operations:RV outflow obstruction(8), pulmonary valve replacement(1), residual VSD(1). 21%(65/304) of patients were followed for >7years(mean:8.8yrs): 2(3%) had severe RV dilation, none had >mild RV outflow obstruction, and all patients had normal RV function except 4(6%) that were mildly depressed.
CONCLUSIONS:
The RVIS strategy has allowed morbidity, mortality, and re-operation rate to be minimized. Most importantly, our midterm results suggest that RVIS does appear to preserve RV function. Longer-term follow-up will be essential in establishing if the RVIS strategy can change the natural history of repaired TOF.