Cardiomyopathies and Myocarditis

Cardiomyopathies are diseases associated with the mechanical and/or electrical dysfunction of the heart muscle (Maron et al. 2006). In children and adolescents, they are often idiopathic, although they may also be complications of CHD, infections, drug toxicity, or genetic disorders (Kipps et al. 2007). The enlarged heart of a dilated cardiomyopathy is the most common cause of heart failure in pediatric patients without other known cardiac defects. Hy-pertrophic cardiomyopathy, a heritable condition marked by thickening of the heart wall, is the leading cause of sudden death in children (Daubeney et al. 2006; DeMaso 2004; Maron et al. 2006).

Myocarditis is an acquired cardiomyopathy that is due to an inflammation of the heart with either an infectious (e.g., viral, bacterial, fungal), parasitic (e.g., Chagas' disease, toxoplasmosis), or autoimmune (e.g., systemic lupus erythematosus, rheumatoid arthritis, acute rheumatic fever, Kawasaki syndrome) etiology (DeMaso 2004; Maron et al. 2006).

Although spontaneous improvement and full recovery are possible, progression to persistent heart failure may occur in severe cases (DeMaso 2004). Cardiomyopathies can result in progressive heart failure, multiorgan dysfunction, arrhythmias, failure to thrive, and/or cardiovascular death (Kipps et al. 2007; Maron et al. 2006). Management of these disorders involves the administration of medications that enhance heart functioning (DeMaso 2004). Approximately 40% of children with symptomatic cardiomyopathies receive a heart transplant or die within the first 2 years of diagnosis (Lipshultz et al. 2003).



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