Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality.

Background: Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.

Methods: A retrospective study was conducted. Patients were included if an initial catheterization was done prior to any intervention. Each patient was assigned an aortic perfusion score based on the amount of antegrade perfusion to the four main coronary arteries. Various characteristics, including aortic perfusion score, were compared between those who required transplant or died during follow-up vs those who did not. Receiver operator curve analysis was done to determine a cutoff point predictive of a composite endpoint of death or transplant.

Results: A total of 64 patients were included in the analysis with 10 reaching the composite outcome. An aortic perfusion score of 227.5 predicted the endpoint with a sensitivity of 90% and a specificity of 83%. For each 1-point increase in the APS, the odds of death or transplant decreased by 1.7%.

Conclusion: The aortic perfusion score can be used to predict a composite endpoint of death or transplant and may be helpful in selecting patients that should be listed for transplant.