Supplement, Optimal Timing of Stage 2 CHSS Fall Work Weekend Jim Meza

% Months since Norwood Poor candidate for Stage 2 7 months 4.8 months  Risk Factor for Death Value After Norwood Birth weight (kg) 2.5 kg MBTS vs. RVPA conduit MBTS Smaller TV diameter (cm) on baseline echo, indexed to BSA 6.92 Aortic valve atresia on baseline echo No After S2P RV dysfunction on pre-S2P echo, moderate or severe Yes Younger age at S2P (days), natural log transformation N/A Weight-for-age z-score on pre-S2P cardiac catheterization -2.1 Required MCS post-Norwood 7 months 4.8 months % 3.5 months 2 months Months since Norwood

% Months since Norwood Poor candidate for Stage 2 Blue, purple = RVPA conduit Green, orange = MBTS  Risk Factor for Death Value After Norwood Birth weight (kg) 2.5 kg MBTS vs. RVPA conduit Smaller TV diameter (cm) on baseline echo, indexed to BSA 6.92 Aortic valve atresia on baseline echo No After S2P RV dysfunction on pre-S2P echo, moderate or severe Yes Younger age at S2P (days), natural log transformation Weight-for-age z-score on pre-S2P cardiac catheterization -2.1 Required MCS post-Norwood RVPA MBTS 7 months 4.8 months 7 months % 4.8 months 3.5 months 3.5 months I would interpret that as – if you are a poor candidate for stage 2 and require intervention at an early age – shunt type does not matter and the survival cost will be very high 2 months 2 months Months since Norwood

% Months since Norwood Patient with more RF for death after Norwood  Risk Factor for Death Value After Norwood Birth weight (kg) 2.8 kg MBTS vs. RVPA conduit MBTS Smaller TV diameter (cm) on baseline echo, indexed to BSA 6.08 Aortic valve atresia on baseline echo Yes After S2P RV dysfunction on pre-S2P echo, moderate or severe No Younger age at S2P (days), natural log transformation N/A Weight-for-age z-score on pre-S2P cardiac catheterization -1.8 Required MCS post-Norwood 2.5 months 2 months % 5.3 months 7 months This figure was for our patient with characteristics that place them at higher risk for death after the Norwood operation (MTS, lower birthweight, +AA). S2P appears it may rescue these patients. Compare with previous slide, who is a poor candidate for CPS, with RV dysfunction and a lower weight for age z-score, who takes a bigger hit for mortality based on younger age at stage 2, and who appars to have a much greater risk for perioperative mortality, especially when younger at tiem of stage 2. Months since Norwood

% Months since Norwood Patient with more RF for death after Norwood Blue, purple = RVPA conduit Green, orange = MBTS  Risk Factor for Death Value After Norwood Birth weight (kg) 2.8 kg MBTS vs. RVPA conduit Smaller TV diameter (cm) on baseline echo, indexed to BSA 6.08 Aortic valve atresia on baseline echo Yes After S2P RV dysfunction on pre-S2P echo, moderate or severe No Younger age at S2P (days), natural log transformation N/A Weight-for-age z-score on pre-S2P cardiac catheterization -1.8 Required MCS post-Norwood RVPA 5.3 months 2.5 months MBTS 7 months 2.5 months % 5.3 months 7 months Months since Norwood