Outcomes After Pediatric Pulmonary Valve Replacement in Patients With Tetralogy of Fallot

Background: People with tetralogy of Fallot might require a trans- annular patch during primary repair, which leads to pulmonary valve regurgitation. Pulmonary valve replacement (PVR) is performed to prevent complications of chronic pulmonary regurgitation, but the optimal timing of PVR remains a matter of debate. In this study we aimed to assess the association of PVR performed in children younger than 18 years of age with the rate of hospitalizations, interventions, and mortality. Methods: This was a retrospective observational cohort of people with tetralogy of Fallot born in Quebec between 1982 and 2015, using a combination of clinical and administrative data. Marginal means and rates models and survival curves were used to compare outcomes of patients with pediatric PVR (younger than 18 years) and those without. Outcomes of interest were rates of cardiac hospitalizations, all-cause hospitalizations, cardiac interventions, and mortality. Groups were balanced using models weighed on the inverse probability of receiving pediatric PVR. Results: Of the 316 eligible patients, 58 (18.4%) received a pediatric PVR. Compared with patients who did not receive pediatric PVR, they were at increased risk of cardiac hospitalizations, although the rates of cardiac hospitalization were low: 0.50 vs 0.09 hospitalizations per 20 years (hazard ratio [HR] = 4.71 [95% confidence interval (CI), 2.229.96]). Patients who received a pediatric PVR had a comparable risk of all-cause hospitalizations (HR = 0.95 [95% CI, 0.71-1.26]) and of cardiac interventions (HR = 1.13 [95% CI, 0.72-1.77]). Conclusions: Patients who underwent pediatric PVR had higher rates of cardiac hospitalizations, but similar rates of all-cause hospitalizations, cardiac procedures, and mortality. In this observational cohort, pediatric PVR was not associated with an improved outcome.