Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy
Authors' conclusion
Our cohort of HLHS patients with systemic RV dysfunction demonstrated a novel finding of improved TFS in those with functional normalization for ≥30 days.
Study context
- Population
- Pediatric hypoplastic left heart syndrome (HLHS) patients with right ventricle (RV) dysfunction lasting >30 days (SickKids heart failure (HF) Database)52% normalized
- Participants
- 99 patients
- Exposure
- Normalization vs no normalization of RV function
- Outcome
- Transplant-free survival (TFS)
- Follow-up
- 2 years after dysfunction onset
Main finding
As reported in the abstract
TFS
P < 0.001
Observational data: associations, not effects.
Further findings
≥1 HF medication at target dosing
P < 0.001
Fontan completion
P < 0.001
Factors associated with improved transplant-free survival (TFS)
- Normalization of function for ≥30 days, onset of dysfunction after bidirectional Glenn, and exposure to ACE inhibition associated with improved TFS
Original publication
Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy. JACC Adv. Published 29 December 2023. doi:10.
Summarised from the published abstract; every number is checked against it