Sunday, May 15, 2022: 2:30 PM - 2:45 PM
15 Minutes
Hynes Convention Center
Room: Room 210
Objective : Tetralogy of Fallot (TOF), pulmonary atresia and major aortopulmonary collaterals (MAPCAs) is a complex congenital heart defect with varied clinical and anatomic findings. For years, our program has recommended early single-staged midline unifocalization at 3 to 6 months of age, in order to maximize the health of the pulmonary microvascular bed. However, many patients are referred beyond 6 months, and it is not clear if outcomes are similar in older infants. Thus, we sought to evaluate surgical outcomes according to age at repair.
Methods : We performed a retrospective chart review to identify patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery for either aorto-pulmonary window creation, unilateral pulmonary artery (PA) origin from a PDA or hemitruncus. Patients were divided in the following age groups: 3.0-4.9months (group 1), 5.0-5.9months (group 2), 6.0-7.9months (group 3) and 8.0-11.9months (group 4). Competing risk regression analyses were performed.
Results : We included 220 patients that were operated from 2001 to 2020. The 4 surgical groups were: 61 patients in group 1, 56 patients in group 2, 56 patients in group 3 and 47 patients in group 4. Baseline characteristics, such as number of MAPCAs (median n=4), genetic syndrome including 22q11 deletion (45% to 52%), preoperative respiratory support (12% to 23%), most often oxygen delivery via nasal cannula, were not different amongst the 4 age groups. Overall, single-stage complete TOF repair with bilateral unifocalization, VSD closure, ASD closure and RV-PA conduit placement was achieved at first surgery in 174 (79%) patients and did not differ across age groups (74% to 84%). Airway-related reoperations, such as aortopexy (n=5, 2%) and tracheostomy (n=7, 3%), as well as duration of post-operative mechanical ventilation (median 4 days) were similar. Early mortality was 4% (n=9) for the entire cohort. At one year following first surgery, 90% of the entire cohort was fully septated. Group 4 was significantly less likely to undergo any catheter-associated PA reinterventions following complete repair (HR 0.44, 95% CI 0.21-0.92, p value 0.028) or surgical PA reinterventions (HR 0.12, 95% CI 0.02-0.95, p value 0.044).
Conclusions : Given the excellent outcomes across all age groups, surgical timing for single-stage unifocalization for TOF-PA-MAPCA should be dictated by clinical and anatomic details, with potential advantage in clinically appropriate older patients who appear to be at a lower probability of PA reinterventions following full septation. Airway complications were not greater in younger infants. Lastly, there was no difference in the likelihood of a single-stage complete repair across the different groups.
8 minute presentation; 7 minute discussion