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22. A Competing Risks Analysis of Outcomes and Associated Factors in 145 Neonates with Tricuspid Atresia: A Congenital Heart Surgeons’ Society Study
Tara Karamlou, David A Ashburn, Christopher A Caldarone, Eugene H Blackstone, Richard A Jonas, Marshall L Jacobs, William G Williams, Ross M Ungerleider, Brian W Mccrindle; Toronto, ON; Canada; Winston-salem, NC; , ON; Canada; Cleveland, OH; Washington, DC; Philadelphia, PA; , ON; Canada; Portland, OR; , ON, Canada

Objective: To identify factors associated with mortality and transition to cavopulmonary connection (CPC) and Fontan procedure in children with tricuspid atresia (TAtr).
Methods: From 1999 to 2004, 145 neonates with TAtr and normally related great vessels were enrolled from first admission to 27 institutions. Antegrade pulmonary blood flow was absent in 20%, restricted 53% and unrestricted in 27%. Important concomitant pathology at admission included mitral valve regurgitation (MR) in 21%, aortic valve pathology or systemic outflow tract obstruction in 12%, and left or bilateral superior vena cavae in 8%. Important MR developed in an additional 23%. Competing risks methodology was used to identify the time-related prevalence and incremental risk factors for death versus CPC, and subsequent death versus Fontan.
Results: For the 145 neonates entered into the study, survival from birth was 96%, 88%, and 86% at 6 months, 3 years and 5 years. Balloon atrial septostomy was performed in 23. Initial palliation included a systemic-pulmonary artery shunt in 66%, PA banding in 11% and CPC in 23%. In-hospital mortality was 4% (n=5) at initial palliation, and occurred following systemic shunt placement. Shunts originating from the innominate artery were most common (66%), and when indexed to body weight (mm•kg^-1) were smaller than those originating from alternate sites (1.15 ± 0.24 vs. 1.31 ± 0.56, P = .07), respectively. Palliated patients who came to CPC without elimination of antegrade pulmonary blood flow (70%) had a tendency toward an increased prevalence of MR compared to those in whom antegrade pulmonary flow was interrupted (23% vs. 7%). Death at any time also occurred more frequently in patients without ligation of the PA at the time of systemic shunt (11 (12%) vs. 3 (3%), P = .16). Competing risks from birth showed that by 1 year of age 86% achieved CPC, 6% died, 7% remain alive without CPC, and 2 patients had single stage Fontan. The median age at CPC was 7 months (range 2 - 23 months). Incremental risk factors for death without CPC included morphologic variables (MR, or a stenotic or regurgitant aortic valve), and procedural variables (palliation with systemic shunt not originating from the innominate artery). CPC occurred earlier and more frequently in those without an initial systemic shunt. Competing risks for events after CPC show that by 3 years 70% had a Fontan, 4% died, and 26% were alive without Fontan.
Conclusions: Mortality prior to cavopulmonary connection is primarily related to the deleterious effects of pulmonary overcirculation. Outcomes can be improved by matching shunt characteristics (size, origin) to specific morphologic patient factors to achieve a balanced circulation.

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