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Interrelationship Between Hemodynamics, Brain Volumes, and Outcome in Hypoplastic Left Heart Syndrome
Bettina Reich1, Kristina N Heye2, Ruth O'Gorman Tuura3
1Pediatric Heart Center, University Hospital Giessen, Justus-Liebig-University, Giessen, Germany.
Insights
Altered hemodynamics in infants with hypoplastic left heart syndrome (HLHS) impact brain growth and neurodevelopment. Higher pressures after stage II correlate with reduced brain volumes and poorer cognitive, language, and motor outcomes.
Area of Science:
- Pediatric Cardiology
- Neurodevelopmental Pediatrics
- Congenital Heart Disease Research
Background:
- Hypoplastic Left Heart Syndrome (HLHS) and Hypoplastic Left Heart Complex (HLHC) present complex congenital heart defects.
- The long-term effects of altered hemodynamics post-stage II palliation on neurodevelopment in HLHS/HLHC patients remain unclear.
- Investigating the association between pre-Fontan hemodynamics and cerebral development is crucial for improving patient outcomes.
Purpose of the Study:
- To determine if elevated central venous and atrial pressures before the Fontan procedure are linked to reduced brain volumes.
- To assess the relationship between pre-Fontan hemodynamics and neurodevelopmental outcomes in children with HLHS/HLHC.
- To compare cerebral volumes in HLHS/HLHC patients with healthy controls.
Main Methods:
- A two-center cohort study included 25 children with HLHS/HLHC undergoing stage II palliation and 8 healthy controls.
- Cerebral MRI scans were analyzed using semiautomated segmentation to determine brain volumes.
- Neurodevelopmental outcomes were assessed using the Bayley Scales of Infant and Toddler Development III (Bayley-III) and neurologic examinations.
Main Results:
- Higher atrial filling pressures in HLHS/HLHC patients correlated with reduced brain volumes and lower language scores.
- Elevated Glenn pressures were associated with increased cerebrospinal fluid, reduced brain volumes, and poorer cognitive, language, and motor scores.
- HLHS/HLHC patients exhibited reduced white matter volumes and increased cerebrospinal fluid compared to controls.
Conclusions:
- Altered cardiovascular hemodynamics following stage II palliation significantly influence brain growth in infants with HLHS/HLHC.
- These hemodynamic changes are associated with impaired neurodevelopmental outcomes, including cognitive, language, and motor deficits.
- Early hemodynamic management may be critical for optimizing neurodevelopmental trajectories in HLHS/HLHC survivors.
Background:
The long-term impact of altered hemodynamics after stage II in children with hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC) on cerebral growth and neurodevelopmental outcome is unknown. We aimed to investigate whether elevated central venous and atrial filling pressures before the Fontan procedure may be associated with smaller brain volumes and poorer neurodevelopmental outcome after the initial hybrid procedure.
Methods:
In a two-center cohort study semiautomated segmentation of cerebral magnetic resonance imaging scans was conducted in 25 children with HLHS/HLHC (25 hybrid) before the Fontan procedure (27.6 ± 4.3 months) and in 8 healthy control subjects (29.7 ± 9.5 months). Study patients were evaluated with the Bayley Scales of Infant and Toddler Development III (Bayley-III) and a neurologic examination. Hemodynamic measures after stage II were assessed with cardiac catheterization at 2 years of age before Fontan completion. Children with known genetic comorbidities were excluded.
Results:
In HLHS/HLHC patients higher atrial filling pressures (6 ± 3 mm Hg; range, 2-14) were correlated with reduced brain volumes and lower language composite score, whereas higher Glenn pressures (10 ± 3 mm Hg; range, 6-16) were related to higher cerebrospinal fluid, reduced brain volumes, and lower cognitive, language, and motoric composite scores in the Bayley-III. Compared with control subjects white matter volumes were reduced and cerebrospinal fluid volumes increased in study patients.
Conclusions:
These data suggest that altered cardiovascular hemodynamics after stage II influence brain growth and neurodevelopmental outcome in infants with HLHS/HLHC.
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