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Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year
Mallory L Hunt1, Richard F Ittenbach2, Michelle Kaplinski3
1Department of Surgery, Division of Cardiovascular Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
Insights
Hospital mortality for hypoplastic left heart syndrome (HLHS) patients undergoing superior cavopulmonary connection (SCPC) has decreased. Length of stay initially improved but plateaued, with complexity impacting outcomes.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Critical Care Medicine
Background:
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect requiring complex surgical palliation.
- The superior cavopulmonary connection (SCPC) is a key stage in surgical palliation for HLHS.
- Optimizing outcomes after SCPC is crucial for improving survival and quality of life in affected children.
Purpose of the Study:
- To estimate hospital mortality and length of stay (LOS) for pediatric patients with HLHS undergoing SCPC.
- To identify trends in mortality and LOS over time.
- To determine risk factors associated with mortality and prolonged LOS after SCPC.
Main Methods:
- Retrospective analysis of HLHS patients who underwent SCPC between 1988 and 2017.
- Stratification of the study period into four eras based on management changes.
- Logistic regression models to identify predictors of mortality and LOS.
Main Results:
- Overall hospital mortality was 4.1% and decreased significantly in later eras.
- Median LOS was 7.0 days, initially decreasing then plateauing.
- Factors associated with mortality included longer support time, younger gestational age, and need for additional procedures.
- Predictors of longer LOS included genetic anomalies, longer prior Norwood LOS, and additional procedures.
Conclusions:
- Hospital mortality following SCPC for HLHS has significantly improved over time.
- Length of stay has shown initial improvement but has plateaued in recent years.
- Patient and procedural complexity, particularly the need for additional interventions, are key determinants of adverse outcomes.
Objectives:
The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC).
Methods:
All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS.
Results:
The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS.
Conclusions:
In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC.
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