Right ventricular infundibulum sparing (RVIS) tetralogy of fallot repair: a review of over 300 patients.

OBJECTIVE

The natural history of standard (large, transmural right ventriculotomy) repair of tetralogy of Fallot (TOF) is associated with a concerning incidence of right ventricular (RV) failure and reoperation. We believe preserving the infundibulum using a TOF repair method with a mini-(<5 mm) 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.

METHODS

Using a retrospective cohort study design, 304 TOF patients who underwent the RVIS strategy (July 1995-June 2008) were reviewed. Median weight and age at repair: 8 kg (3-62 kg) and 9 months (2 days-23 years). Seventeen percent (51) of patients required a systemic-to-pulmonary artery shunt.

RESULTS

Ninety-nine percent of patients had a mini- 73% (222) or no 26% (79) ventricular incision. Postoperative morbidity included arrhythmias 3% (10), postoperative bleeding 2% (7), temporary renal failure 1% (3), and neurologic injury <1% (2). Thirty-day survival was 99.7%. Overall 1 and 7-year Kaplan-Meier survivals were 97% and 96%. In nonsyndromic children, only 1 patient has died in the RVIS strategy. A total of 3.2% (10) of patients had reoperations. Twenty-one percent (65/304) of patients have been followed for >7 years (median: 8.5 years). None of them have severe dilation, > mild RV outflow obstruction, an arrhythmia, or a pacemaker/AICD. Ninety-five percent of these patients have normal RV function; 3 (4.6%) had mild dysfunction. This cohort has excellent exercise tolerance (MaxVO2 (mean): 41 ± 12 mL/kg/min).

CONCLUSION

The RVIS strategy has allowed morbidity, mortality, and reoperation rates to be minimized. 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.