Respiratory failure and cardiac disturbances in myotonic dystrophy

Cardiopulmonary abnormalities are frequently encountered in myotonic dystrophy. We present five patients with myotonic dystrophy who entered the intensive care unit in acute respiratory failure. The possible etiologic factors of pulmonary complications are reviewed. The most important is probably aspiration pneumonia. The difficulties in the treatment of the respiratory failure are emphasized. Myotonia of the chest muscles and diaphragm make artificial ventilation difficult. Recovery is delayed chiefly by swallowing disturbances. General anaesthesia is hazardous. Four patients presented cardiac arrhythmias and/or conduction abnormalities which were transient.