High Risk Status for Stage I Palliation Increases Mortality After Stage II But Not Stage III

High risk (HR) factors have been shown to have increased rates of mortality after stage 1 palliation (S1P) for single ventricle physiology. It remains unclear how initial HR status affects longitudinal outcomes after subsequent stage 2 palliation (S2P) and stage 3 palliation (S3P).

Single ventricle patients undergoing S1P between July 2004 and October 2018 at a single institution were included. Patients having one or more HR factors were considered to have HR status, with all others classified as low risk (LR). Longitudinal survival stratified by risk status was compared after each palliative stage, in addition to readmission and length of stay. Proportional hazards modeling was used to determine risk factors for longitudinal mortality.

Of 132 patients presenting during the study for S1P, 57 (43.2%) were classified as HR. Overall 10-year survival was decreased in the HR cohort (P = .001). The HR patients were at significantly increased risk of death during interstage I (P = .01) and interstage II (P = .01), but survival was similar to that of LR patients after S3P (P = .31). Readmission rates after S2P were higher among HR patients (41.9% vs 22.5%, P = .029), but were similar after S3P. Length of stay was increased in the HR cohort after S2P (median 11 vs 9 days, P = .024) but similar to the LR group after S3P. Prematurity was the risk factor most consistently associated with increased mortality after all stages.

A high risk status of patients undergoing S1P portends a higher risk of mortality, length of stay, and readmission after S2P. High-risk patients have survival similar to that of low-risk patients after S3P.