Relevance of Metabolic Profiles in Children With Cardiac Disease
Abstract
Fat is primarily a way to store energy. Fat in the form of fatty acids combines with carnitine to form acylcarnitines and these are transported into the cell’s mitochondria where the fatty acids are released and metabolised to provide energy. Acylcarnitines can be measured in blood and this reflects concurrent fat metabolic status. This acylcarnitine test, along with several amino acids and endocrine markers is performed in all babies born in New Zealand as part of a routine newborn screening test enabling diagnosis of rare genetic metabolic disorders. This data is stored in the newborn metabolic screening laboratory database at Auckland City Hospital. The heart has high energy requirements and fat is the main fuel. The failing heart results in disruption to fatty acid metabolism. It has been recently reported that adults with heart failure have plasma acylcarnitine profiles that differ from the normal population with higher levels of long chain fatty acid derivatives. Babies with severe congenital heart disease can present to hospital in the first two weeks of life. Most of these cases are seen at Starship Children’s Hospital in Auckland. Many of these babies will have stressed myocardium. It is possible that these babies will, like their adult counterparts, have abnormal fat metabolism that can be demonstrated by an abnormal acylcarnitine profile. In addition to congenital heart lesions, the acylcarnitine profiles of children with cardiomyopathy were also reviewed. These…
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Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence.
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