Long-term progression and survival following Norwood Single Ventricle Reconstruction

Much data exist concerning Norwood discharge mortality. Less is known about late survival. Examining the available data in light of the Single Ventricle Reconstruction trial is insightful as focus shifts toward long-term survival.

Data from 2000 to 2001 demonstrated approximately 40-50% 10-year survival, 30-40% or less between 10 and 15 years. The shape of the curves was characteristic; the majority of deaths within the first year, followed by a late constant phase. Publications from 2001 to 2005 suggested that various combinations of technical and perioperative modifications allowed hospital discharge survivals as high as 90-94%. As results matured (2005-2010) a consistent message was that, although the shape of the newer curves was similar (highest hazard in the first 1 year), higher hospital survival shifted the later phase to yield better long-term survival (70-85% between 5 and 10 years). Some emphasized right ventricle-based shunts as a 'cause' of improving results. Since 2010, the Single Ventricle Reconstruction trial has matured and has increasingly shifted opinion away from the right ventricle shunt as a 'cause' of improved results. The survival of the right ventricle shunt group is slightly higher at 3 years, but the 1-year statistical significance has been lost and the two groups converge. As the Single Ventricle Reconstruction study was based on the interaction between randomized shunt and survival, the secondary and other endpoint analyses must be cautiously considered.

The current English-language literature suggests a 60-80% 5-10 year survival expectation. The shape of the survival curve remains; the highest hazard remains the first year before a later, stable phase is reached. Rather than a 'magic bullet' theory surrounding one technique or practice, centers have differentially adopted various combinations to optimize Norwood survival. Optimizing interstage I survival is a challenge to further increase the percentage of patients reaching the late, stable phase.