Matching procedure to morphology improves outcomes in neonates with tricuspid atresia

Competing risks depiction of events after diagnosis in 150 patients with tricuspid atresia. All patients began alive and thereafter migrated to one of four mutually exclusive end states (death, BDCPA, single-stage Fontan completion, or remaining alive without BDCPA) at time-dependent rates defined by underlying hazard functions. At any point in time, sum of proportions of children in each state is 100%. For example, estimated prevalences after 2 years from diagnosis are as follows: 89% BDCPA; 6% dead without BDCPA, 4% alive without BDCPA, and 1% single-stage Fontan completion. Solid lines represent parametric point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate estimated proportions of patients in each state at 2 years from diagnosis.

Mortality without BDCPA stratified by shunt origin. Patients who had systemic-pulmonary arterial shunts originating from innominate artery had significantly improved survival relative to those with shunts from subclavian artery or other sites, which carried nearly equivalent mortality rates.

Smaller systemic-pulmonary arterial shunt size resulted in decreased mortality and increased transition rates to BDCPA. A, Use of 3-mm shunt resulted in 85% of patients having BDCPA by 1 year, 10% still in palliated state, and only 5% of dead. B, Use of larger 5-mm shunt resulted in much slower transition rate and slightly increased rate of death without BDCPA.

Competing-risks depiction of events after BDCPA in 128 patients who underwent BDCPA. Estimated prevalences after 3 years from BDCPA are as follows: 75% Fontan completion, 20% alive without Fontan conversion, and 5% dead without Fontan completion. Solid lines represent parametric point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate estimated proportions of patients in each state at 3 years from BDCPA.

Proportion of patients discharged alive after BDCPA stratified by patient age at BDCPA. Of 128 patients undergoing BDCPA, 126 were discharged alive. Multivariable competing risks equation for survival to hospital discharge was solved for hypothetic patient weighing 3.7 kg with previous palliation at three different values for age at BDCPA. Age at BDCPA did not influence either hospital LOS or in-hospital mortality. Youngest patients in our study undergoing BDCPA (at about 2 months) fared no worse than did those of median or older age.

Flow chart depicting events from diagnosis among 150 patients with tricuspid atresia. One patient enrolled during 2004 remains alive without operation. All other patients underwent palliation consisting of systemic-pulmonary arterial shunt, PA banding, or initial BDCPA. Nine deaths occurred after systemic-PA shunt placement, and 128 patients had BDCPA. Of those who had BDCPA, 7 died: 6 before Fontan completion and 2 after Fontan conversion. Seventy-four patients underwent staged Fontan completion. Two patients had single-stage Fontan completion after systemic-pulmonary arterial shunt placement.

Overall time-related survival among 150 patients with tricuspid atresia. Solid lines represent parametric point estimates enclosed by 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses represent number of patients traced at that point.

A, Predicted mortality without achievement of BDCPA stratified by systemic-pulmonary arterial shunt origin in absence of mitral valve regurgitation (MR). B, Predicted mortality without achievement of BDCPA stratified by systemic-pulmonary arterial shunt origin in presence of mitral valve regurgitation. Although non–innominate artery shunt origin results in decreased survival in both conditions, its unfavorable influence is greater in presence of significant mitral regurgitation. Solid lines represent continuous parametric estimates.

Predicted achievement of and transition rates to BDCPA from diagnosis stratified by type of palliation and size of initial systemic-pulmonary arterial shunt. Five lines represent five specific solutions to multivariable equation for achievement of BDCPA for hypothetic patient without additional anomalies admitted at 1 year of age. Mean values were used for other continuous predictors. For patients requiring systemic-pulmonary arterial shunt, placement of smaller diameter shunt is associated with greatest prevalence of patients reaching defined end state. Solid lines represent continuous parametric point estimates.

Competing risks diagram of events after BDCPA to hospital discharge. Median LOS was 6 days (range 3-351 days). Competing risks analysis showed that 60 days after BDCPA, 95% were discharged alive, 4% remained alive in hospital, and 1% died without discharge.