Glycogenosis type IV as a seldom cause of cardiomyopathy - report about a successful heart transplantation]

We report about a 17 year old male patient with a cardiomyopathy secondary to type IV glycogenosis (Andersens disease) and class II immunoglobulin deficiency who underwent cardiac transplantation.The patient first developed symptoms of heart failure at the age of twelve. The histologic diagnosis was cardiomyopathy secondary to glycogenosis. In addition, the patient suffered recurrent pulmonary infections and developed bronchiectases in the left lower lobe. This region was atelectatic since he was eleven.The patient did have two younger brothers who died of congestive heart failure at the age of nine and ten. Neither his parents nor anybody else of his relatives had a history of heart failure or glycogenosis. Since the patient suffered recurrent cardiac decompensations with the need for catecholamines he was accepted for cardiac transplantation although several relative contraindications to transplantation such as cachexia, myopathy, immunglobulin deficiency and bronchiectases had been present. The patient was transplanted successfully. The postoperative weaning from the respirator was markedly prolonged and complicated by pulmonary infection. Furthermore, mobilization was retarded. One year after transplantation, he is in a good condition without pulmonary or systemic infection. Right ventricular endomyocardial biopsies did not show recurrence of glycogenosis in the donor organ.