Mid-term results of correction of Tetralogy of Fallot with absent pulmonary valve

Background: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Methods: We performed a retrospective review of 73 consecutive patients who underwent repair for TOF/APV between January 2005–August 2015. Mean age was 6.4 ± 5.6 years (28 days–22 years). The right ventricular outflow tract (RVOT) was reconstructed using varied techniques. Freedom from RVOT gradients and re-operation was studied. Results: There were four (5.5%) early deaths, two each in infants and older children. Median ICU stay was 2 days (range, 1–12 days). Mean ICU stay for, infants, children and adults, was 6.5 ± 6.04, 2.75 ± 2.45, and 2.33 ± 1.03 days, respectively (p = 0.0762). Median hospital stay was 6 days (range, 4 to 15 days). Mean hospital stay for, infants and children and adults was 7 ± 2, 6.75 ± 2.39, and 6.33 ± 1.63 days, respectively (p = 0.325). Mean follow up was 65 ± 36.6 months (median 56 months, range 7–126 months). On follow up echocardiography, 14 (21.21%) had no pulmonary regurgitation. 21 (31.81%) had mild PR patients, 8 (12.12%) moderate PR and 19 (28.78%) had severe PR. There were five (7.5%) reoperations. Five and ten-year survival was 95% ± 2.12 and 92.3% ± 3.45 respectively. Freedom from RVOT reoperation was 93 ± 2.62% and 89 ± 3.87% at 5 and 10 years. Conclusions: In contrast to children and adults with TOF/APV, infants carry significant early mortality. But the mid-term outcome for patients who survive the initial repair of TOF/APV is acceptable. However, these patients require constant surveillance and irrespective of the methods of RVOT management, the reoperation rates are expected to be high as more of these patients survive into adulthood