101. Infants of all Age Undergoing Single-Staged Midline Unifocalization are associated with Excellent Outcomes

*Osami Honjo Invited Discussant
Labatt Family Heart Centre, Hospital for Sick Children
Toronto, ON 
Canada
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Dr. Osami Honjo is a staff cardiovascular surgeon in The Hospital for Sick Children, adult congenital cardiovascular surgeon in Toronto General Hospital, and a professor of surgery in the University of Toronto. Dr. Honjo is the surgical director of heart transplantation and mechanical circulatory support, and a senior associate scientist in Translational Medicine in the Research Institute. Dr. Honjo holds the Watson Family Chair in Cardiovascular Science since 2019. Dr. Honjo specializes in neonatal and complex paediatric cardiac surgeries, surgeries for single ventricles, and mechanical circulatory support and heart transplantation. Dr. Honjo obtained multiple national research grants for ex-vivo heart perfusion and development of mechanical support for failing single ventricle patients, and has authored and co-authored more than 190 scientific publications.

Elisabeth Martin Abstract Presenter
Lucille Packard Children's Hospital
Stanford
United States
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Elisabeth Martin MD MPH, clinical assistant professor in pediatric and congenital heart surgery at Lucile Packard Children's Hospital, Stanford University. Special interest for complex pulmonary artery reconstruction, including patients with Williams and Alagille syndromes, as well as unifocalization for pulmonary atresia, VSD and MAPCAs. 

Sunday, May 15, 2022: 2:30 PM - 2:45 PM
15 Minutes 
Hynes Convention Center 
Room: Room 210 

Abstract

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.

Presentation Duration

8 minute presentation; 7 minute discussion