Early onset of complete heart block in Pearson syndrome

  title={Early onset of complete heart block in Pearson syndrome},
  author={Shamima Rahman and James V. Leonard},
  journal={Journal of Inherited Metabolic Disease},
A 5-year-old girl with Pearson marrow pancreas syndrome (McKusick 557000) presented following a sudden episode of apnoea. She was pale, cyanosed and bradycardic, with a heart rate of 20 beats/min and metabolic acidosis (pH 7.2, bicarbonate 11, base de¢cit 15 mmol/L). The heart rate increased from 20 to 45 after resuscitation with intravenous £uids and bicarbonate. ECG demonstrated complete atrioventricular block, with atrial rhythm at 120 beats/min and idioventricular rhythm at 45 beats/min… CONTINUE READING


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