Elective primary repair of acyanotic tetralogy of Fallot in early infancy: overall outcome and impact on the pulmonary valve

OBJECTIVES We sought to determine if early primary repair of acyanotic tetralogy of Fallot (ToF) can be performed safely with low requirement for transannular patching (TAP) and thereafter allow normal right ventricular outflow tract (RVOT) growth. BACKGROUND Early primary repair of ToF normalizes intracardiac flow patterns, which may allow subsequent normal RVOT growth. Traditionally repair is deferred until symptoms occur or children are deemed of adequate size for operative risk to be acceptable because of a perceived increased requirement for TAP in small infants. METHODS Between July 1992 and March 1999, 42 acyanotic infants aged 4 to 87 days (median 62) and weight 2.6 to 6.6 kg (median 4.55) underwent complete repair of ToF. Pulmonary annulus measured 4 to 10.5 mm (median 6.5) with “z-value” of −5.6 to +3.0 (median −1.9). RVOT reconstruction was tailored to each patient; pulmonary valvotomy was performed in 26, main pulmonary arterioplasty in 22, and infundibular patching in 2. Only 10 (24%) required TAP. RESULTS Postoperative RVOT gradient was 0 to 30 mm Hg (median 10) and pRV/pLV ratio 0.3 to 0.6 (median 0.44). Pulmonary insufficiency was trivial/mild. There were no deaths. Junctional ectopic tachycardia developed in seven; only one required treatment. ICU stay was 2 to 14 days (median 4) and hospital stay 4 to 22 days (median 7). At follow-up 12 to 64 months later (median 38) there were no deaths. One child required reoperation for recurrent RVOT obstruction and two required balloon pulmonary arterioplasty. Follow-up RVOT gradient was 0 to 36 mm Hg (median 12), unchanged from early postoperative condition, and median z-value was −1.2 (−2.8 to +2.5); pulmonary insufficiency remained trivial/mild. CONCLUSIONS Complete repair of acyanotic ToF can be performed in early infancy with low morbidity and mortality and low requirement for TAP. Though results are not statistically significant, early repair may allow normal RVOT growth thereafter