Kardiomyopathien im Kindesalter*

: Primary cardiomyopathies are heart muscle diseases of unknown cause. In hypertrophic cardiomyopathy (HCM) a hypertrophy of unknown aetiology results either in asymmetric septal hypertrophy (mostly with obstruction) or in concentric hypertrophy of the left ventricle (mostly without obstruction). This condition is rare in childhood, partly inherited and sometimes observed already in newborns. Sudden cardiac death is a main complication in the second decade. In non-obstructive HCM endomyocardial biopsy is the tool to differentiate secondary forms. Dilated cardiomyopathy is recognized by aetiologically unclear dilatation of left or right or both ventricles. In childhood this condition has to be differentiated from coronary anomalies, endocardial fibroelastosis, and myocarditis. Also rare secondary forms have to be considered because of possible therapeutic consequences. From the very few histologically proven reports a reliable prognosis for this disease in childhood cannot be deduced.

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