Catecholamine toxicity in aromatic L-amino acid decarboxylase deficiency.

Abstract

This report presents the case of an adult male with aromatic L-amino acid decarboxylase deficiency who developed serious cardiac rhythm disturbances during treatment with intravenous dopamine and norepinephrine for severe hypotension. Three weeks later, he spontaneously developed atrial fibrillation while not receiving exogenous catecholamines. He died suddenly after several months. We presume cardiac arrhythmia was the most likely cause of his death. Patients with aromatic L-amino acid decarboxylase deficiency may be prone to cardiac arrhythmias at rest and also may be exceptionally sensitive to exogenous catecholamines. Therefore, close cardiac monitoring is indicated at baseline and during treatment with pressors.

• Authors: Irina A Anselm, Basil T Darras
• Journal: Pediatric neurology (Pediatr Neurol) Vol. 35 Issue 2 Pg. 142-4 (Aug 2006) ISSN: 0887-8994 [Print] United States
• PMID: 16876014 (Publication Type: Case Reports, Journal Article)

Chemical References

• Cardiotonic Agents
• Vasoconstrictor Agents
• Aromatic-L-Amino-Acid Decarboxylases
• Dopamine
• Norepinephrine

Topics

• Adult
• Aromatic-L-Amino-Acid Decarboxylases (deficiency)
• Arrhythmias, Cardiac (etiology)
• Cardiotonic Agents (adverse effects)
• Dopamine (adverse effects)
• Fatal Outcome
• Humans
• Male
• Norepinephrine (adverse effects)
• Vasoconstrictor Agents (adverse effects)