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Plasma amino acids in interrupted aortic arch and the hypoplastic left heart syndrome
Insights
Infants with congenital heart disease, specifically interrupted aortic arch (IAA) and hypoplastic left heart syndrome (HLH), show elevated plasma amino acids. These levels differ between IAA and HLH, potentially linked to metabolic acidosis and liver function.
Area of Science:
- Biochemistry
- Pediatric Cardiology
- Neonatology
Background:
- Congenital heart disease (CHD) significantly impacts infant physiology.
- Left-sided obstructive lesions, such as interrupted aortic arch (IAA) and hypoplastic left heart syndrome (HLH), present unique challenges.
- Understanding metabolic alterations in neonates with CHD is crucial for management.
Purpose of the Study:
- To investigate plasma amino acid profiles in neonates with left-sided congenital heart disease.
- To compare amino acid levels between infants with IAA and HLH.
- To explore potential correlations between amino acid levels, acid-base status, and specific CHD types.
Main Methods:
- Plasma amino acid concentrations were measured in 8 neonates (3 IAA, 5 HLH) within the first week of life.
- Levels were compared to a control group of 40 healthy infants matched for gestational age and birth weight.
- Blood pH was assessed at the time of sampling.
Main Results:
- Total plasma amino acid levels were significantly higher in infants with left-sided CHD compared to controls.
- Both branched-chain and aromatic amino acids were elevated.
- Infants with HLH exhibited higher total amino acid levels than those with IAA, primarily due to increased branched-chain amino acids. HLH infants also presented with metabolic acidosis, unlike IAA infants.
Conclusions:
- Elevated plasma amino acids are observed in neonates with obstructed left-sided congenital heart disease.
- Differences in amino acid profiles exist between IAA and HLH, potentially influenced by metabolic acidosis and hepatic hypoperfusion.
- These findings suggest that altered hepatic function and acid-base balance play a role in the metabolic derangements associated with severe left-sided CHD.
Abstract:
Plasma amino acids were measured in 8 babies with left-sided congenital heart disease, 3 interrupted aortic arch (IAA) and 5 hypoplastic left heart syndrome (HLH), at 1-6 days of age. There was an increase in total amino acid levels when compared with 40 control infants of similar gestational age and birth weight. Both branched-chain and aromatic amino acids were raised although there were differences between the babies with IAA and HLH. Babies with HLH had higher total amino acid levels than babies with IAA, the difference being due to raised branched-chain amino acids. Apart from the anatomical differences babies with HLH had metabolic acidosis at the time of sampling whereas babies with IAA had normal pH. It is possible that the increase in amino acid levels in babies with obstructed left-sided congenital heart disease is due to hepatic congestion and hypoperfusion, although altered acid base states may also be important.