GM1 Gangliosidose und dilatative Cardiomyopathie

: We report a child with a rare cardiac involvement in GM1 gangliosidosis. At the age of 9 months a secondary dilatative cardiomyopathy due to the metabolic disorder was diagnosed. During 2 years the functional echocardiographic signs became normal, though the ECG still was not normal. Our report is the first longterm observation and investigation of cardiac involvement in GM1 gangliosidosis.