Cumulative comorbid conditions influence mortality risk after staged palliation for hypoplastic left heart syndrome and variants

J Thorac Cardiovasc Surg. 2023 Jan;165(1):287-298.e4. doi: 10.1016/j.jtcvs.2022.01.056. Epub 2022 Apr 2.

Abstract

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.

Keywords: 1-year survival; Norwood; genetic syndromes; high-risk diagnoses; hybrid palliation; hypoplastic left heart syndrome; low birth weight; prematurity; stage 1 palliation.

Publication types

  • Multicenter Study

MeSH terms

  • Child
  • Humans
  • Hypoplastic Left Heart Syndrome*
  • Norwood Procedures* / adverse effects
  • Palliative Care
  • Prospective Studies
  • Retrospective Studies
  • Risk Factors
  • Treatment Outcome