Catalog

Hypertrophic cardiomyopathy due to disorder of fatty acid metabolism

No MMS code

Link to WHO Definition

Hypertrophic cardiomyopathy due to disorder of fatty acid metabolism – http://id.who.int/icd/entity/1868316982

Hypertrophic cardiomyopathy due to disorder of fatty acid metabolism is the presence of hypertrophic cardiomyopathy in association with a disorder of the fatty acid metabolism, including disorders of fatty acid uptake and transport (such as carnitine transport defects) and very long-chain acyl-CoA dehydrogenase deficiency. Characteristic is hypoglycaemia with low ketone levels due to an inability to convert fatty acids into ketones (hypoketotic hypoglycaemia), in association with low insulin levels.