A more sophisticated PRAiS2 risk model for UK use was developed with additional comorbidity and diagnostic information, alongside age and weight as nonlinear variables.
ObjectiveTo describe the long term outcomes, treatment pathways and risk factors for patients diagnosed with Hypoplastic Left Heart Syndrome (HLHS) in England and Wales.
MethodsThe UKs national audit database captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales. HLHS patients born between 2000 and 2015 were identified using codes from the International Paediatric and Congenital Cardiac Code.
ResultsThere were 976 patients with HLHS. Of these, 9.6% had a pre-pathway intervention, 89.5% underwent a traditional pathway of staged palliation and 6.4% of infants underwent a hybrid pathway. Patients undergoing pre-pathway procedures or the hybrid pathway were more complex, exhibiting higher rates of prematurity and acquired comorbidity. Pre-pathway intervention was associated with the highest in-hospital mortality (34.0%).44.6% of patients had an off pathway procedure after their primary procedure, most frequently stenting or dilation of residual or re-coarctation and most commonly occurring between Stage 1 and 2.The survival rate at 1 year and 5 years was 60.7% (95% CI 57.5-63.7) and 56.3% (53.0-59.5) respectively. Patients with an antenatal diagnosis (multivariable hazard ratio (MHR) 1.63 (95% CI 1.12-2.38)), low weight (<2.5kg) (MHR 1.49 (1.05-2.11)) or the presence of an acquired comorbidity ) were less likely to survive.
ConclusionTreatment pathways amongst HLHS patients are complex and variable. It is essential that the long term outcomes of conditions like HLHS that require serial interventions are studied to provide a fuller picture and to inform quality assurance and improvement.
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KEY MESSAGES
In an era of low mortality rates across a wide range of operations, non-procedure-based risk factors form a vital element of risk adjustment and their presence leads to wide variations in the predicted risk of a given operation.
supporting Libby Rogers. The remaining authors have disclosed that they do not have any potential conflicts of interest.derive scores for the first 12 hours after surgery. The IDO2 Index was calculated by Etiometry using vital signs and laboratory data. A modified LCOSS (mLCOSS) was calculated from clinical and therapeutic markers. The mean IDO2 and mLCOSS were compared within each matched pair using the Wilcoxon signed-rank test. IDO2 correctly differentiated adverse events in 13/28 matched pairs, with no evidence of IDO2 being higher in cases (p=0.71). mLCOSS correctly differentiated adverse events in 23/28 matched pairs, with strong evidence of a raised score in LCOS cases (p<0.01).
ConclusionsAlthough IDO2 is an FDA approved indicator of risk for low mixed venous oxygen saturation, early post-operative average values were not linked with medium term adverse events. The indicators included in the mLCOSS had a much stronger association with the specified adverse events.
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