Tetralogy of Fallot with absent pulmonary valve syndrome (ToF-APVS) is a rare variant of tetralogy, associated with severe pulmonary valve regurgitation and aneurysmal dilation of the pulmonary arteries (PAs). Reported outcomes after surgical corrections are limited to single center, older series and might not reflect the current outcome. We aim to use data from a national registry to evaluate short- and long-term outcomes after surgical repair of ToF-APVS, to serve for counselling and planning. All children undergoing ToF-APVS repair in the UK between 2002 and 2013 were included. Survival and freedom from reintervention were estimated using the Kaplan-Meier method, and univariable analysis was done using the Weibull regression model. A total of 98 children, 45% male, 10% with DiGeorge syndrome, median age of 213 days (1 day to 13 years) were included. Mortality at 30 days was 3.3%, higher for neonates (6.7% vs 2.7%, P = 0.4) and those on preoperative mechanical ventilatory support (16.7% vs 1.3%, P = 0.04). Survival was 92.1% and freedom from pulmonary valve or conduit replacement (PVR) 73.2% at 10 years. Neonates had worse survival (hazard ratio [HR] 6.2, P = 0.02), freedom from PVR (HR 4.5, P = 0.01), freedom from PAs arterioplasty (HR 6.6, P = 0.001), and overall freedom from any reintervention (HR 5.3, P < 0.001). Low weight at repair was associated with worse freedom from PVR (P = 0.02) and from PAs arterioplasty (P = 0.009), preoperative ventilatory support with increased mortality (P = 0.009), the presence of DiGeorge syndrome was associated with worse freedom from PVR (HR 4, P = 0.02). Surgical repair of ToF-APVS can be performed with low early and late mortality, with improving results even in those with preoperative mechanical ventilatory support. The need for right ventricular outflow tract reintervention, including on the PAs, is an expected issue in the long term for the majority of patients.
- 22q11.2 deletion
- Pulmonary arterioplasty
- Pulmonary valve replacement
- Tetralogy of Fallot absent valve