Biomedical subjects
Publications and source records attributed to I Hammerer.
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.
PS is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and courses between trachea and esophagus to the hilus of the left lung causing compression of the right mainstem bronchus and trachea. In about half of all cases this vessel anomaly is associated with tracheo-bronchial and/or cardiovascular malformations. We present 6 patients with PS concentrating on the accompanying malformations of the tracheobronchial tree. All patients had the typical symptoms of wheezing and stridor already in early infancy. 3 patients showed concomitant tracheobronchial anomalies. 2 patients had additional cardiovascular deformities. In 3 of the patients the positive clinical course allowed conservative therapy. In the case of 2 further patients the respiratory problems demanded surgery, one of this patients died. A further patient died after palliative surgery of cardiac anomaly.