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Cumulative comorbid conditions influence mortality risk after staged palliation for hypoplastic left heart syndrome
Emily R Backes1, Natasha S Afonso1, Danielle Guffey1
1Divisions of Cardiology and Critical Care, Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Tex, Dan L. Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, Tex.
Insights
High-risk conditions like prematurity and genetic syndromes impact infant survival after stage 1 palliation. Multiple high-risk diagnoses significantly reduce survival odds to the first birthday.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Quality Improvement
Background:
- Stage 1 palliation is critical for infants with complex congenital heart disease.
- Certain conditions (prematurity, genetic syndromes, extracardiac issues) are known high-risk factors for mortality.
- Understanding the cumulative impact of these conditions is vital for improving outcomes.
Purpose of the Study:
- To evaluate the impact of high-risk conditions on survival to the first birthday after stage 1 palliation.
- To quantify the cumulative burden of multiple high-risk diagnoses on infant outcomes.
- To identify specific factors associated with mortality in this vulnerable population.
Main Methods:
- Prospective data from the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry.
- Categorization and quantification of comorbid high-risk conditions in 1421 participants.
- Logistic regression analysis to determine factors associated with mortality.
Main Results:
- 40% of participants had at least one high-risk condition, with lower survival (76.2%) compared to standard risk (88.1%).
- A single high-risk diagnosis did not significantly reduce survival, but multiple diagnoses showed a dose-dependent decrease in survival odds.
- Extracorporeal membrane oxygenation support and cardiac reoperation post-palliation were associated with reduced survival odds.
Conclusions:
- One high-risk diagnosis alone does not significantly impact 1-year survival after stage 1 palliation.
- The cumulative number of high-risk diagnoses is strongly associated with decreased survival odds.
- Further research with larger patient cohorts is needed to analyze specific comorbid conditions.
Objective:
Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative.
Methods:
The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried. Comorbid conditions were categorized and quantified to determine the cumulative burden of high-risk diagnoses on survival to the first birthday. Logistic regression was applied to evaluate factors associated with mortality.
Results:
Of the 1421 participants, 40% (575) had at least 1 high-risk condition. The aggregate high-risk group had lower survival to the first birthday compared with standard risk (76.2% vs 88.1%, P < .001). Presence of a single high-risk diagnosis was not associated with reduced survival to the first birthday (odds ratio, 0.71; confidence interval, 0.49-1.02, P = .066). Incremental increases in high-risk diagnoses were associated with reduced survival to first birthday (odds ratio, 0.23; confidence interval, 0.15-0.36, P < .001) for 2 and 0.17 (confidence interval, 0.10-0.30, P < .001) for 3 to 5 high-risk diagnoses. Additional analysis that included prestage 1 palliation characteristics and stage 1 palliation perioperative variables identified multiple high-risk diagnoses, poststage 1 palliation extracorporeal membrane oxygenation support (odds ratio, 0.14; confidence interval, 0.10-0.22, P < .001), and cardiac reoperation (odds ratio, 0.66; confidence interval, 0.45-0.98, P = .037) to be associated with reduced survival odds to the first birthday.
Conclusions:
The presence of 1 high-risk diagnostic category was not associated with decreased survival at 1 year. Cumulative diagnoses across multiple high-risk diagnostic categories were associated with decreased odds of survival. Further patient accrual is needed to evaluate the impact of specific comorbid conditions within the broader high-risk categories.
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