Congenital heart disease

Increased Endothelin-1 Is Associated With Morbidity in Single Ventricle Heart Disease in Children Undergoing Fontan Palliation

JACC AdvPublished prospective cohort study

Authors' conclusion

SVHD children with higher peri-operative ET1 experience more post-stage 3 morbidity. Failure to suppress ET1 may be a modifiable risk factor for intolerance of SVHD palliation.

Study context

Population
Children with single ventricle heart disease (SVHD) undergoing stage 3 (Fontan) palliation, and controlschildren with SVHD undergoing stage 3 palliation 84 · controls 50
Participants
84 vs 50
Exposure
Persistent failure to suppress ET1 and impaired recovery among children with SVHD undergoing the stage 3 (Fontan) operation
  • SVHD casesn = 84
  • Controlsn = 50
Outcome
Fontan pressure and systemic oxygen saturation
Follow-up
24 hours postoperation

Main finding

As reported in the abstract

  • Adjusting for clinical covariates, higher preoperative ET1 was associated with higher 24-hour Fontan pressure
  • Higher 24-hour postoperative ET1 was associated with lower systemic oxygen saturation at 24 hours postoperation and higher 24-hour Fontan pressure
  • Adjusting for clinical covariates

Observational data: associations, not effects.

Further findings

ET1 in systemic vein, SVHD cases vs controls · lower in SVHD cases

P = 0.001

OutcomeP value
ET1, systemic vein vs pulmonary vein (SVHD cases) · preoperationHigher in systemic veinP < 0.001
  • ET1, systemic vein vs pulmonary vein (SVHD cases): systemic vein 1.0 pg/mL
  • ET1, systemic vein vs pulmonary vein (SVHD cases): pulmonary vein 0.7 pg/mL

ET1 time course among cases · 2, 24 and 48 hours postoperation

ET1 concentration peaked at 2 hours postoperation, decreased by 24 hours, and was stable but not back to baseline by 48 hours

Pleural drainage and length of stay

Higher 24-hour postoperative ET1 was associated with more pleural drainage and longer length of stay

Original publication

Increased Endothelin-1 Is Associated With Morbidity in Single Ventricle Heart Disease in Children Undergoing Fontan Palliation. JACC Adv. Published 20 March 2025. doi:10.1016/j.jacadv.2025.101672

Summarised from the published abstract; every number is checked against it

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