ABSTRACT
This discussion of inherited metabolic causes of pediatric cardiac failure will encompass processes affecting energy production, and the storage diseases that affect cardiac systolic and=or diastolic function. Several diseases have a component of cardiac hypertrophy without cardiac failure in the presentation (Table 1). Only those with clear cardiac pathology are included. Metabolic derangements can cause arrhythmogenesis either associated with, or as a result of, cardiac failure, and these will be noted. Genetic abnormalities of contractile
force generation and transmission, now thought to be the basis of many types of idiopathic hypertrophic and dilated cardiomyopathy, fall out of the scope of this discussion of altered myocardial metabolism. Recent reviews of the point mutations and deletions that affect the cytoskeletal and sarcomere structure and function in inherited hypertrophic and dilated cardiomyopathies are available (1-5). An exhaustive database of these gene defects is available on the web site http:==www.angis.org.au.
