Catecholamine toxicity in aromatic L-amino acid decarboxylase deficiency.
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.
Anselm
Irina A
IA
Department of Neurology, Children's Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA. Irina.Anselm@childrens.harvard.edu
Darras
Basil T
BT
D000328:Adult; D001142:Aromatic-L-Amino-Acid Decarboxylases / Q000172:deficiency*; D001145:Arrhythmias, Cardiac / Q000209:etiology*; D002316:Cardiotonic Agents / Q000009:adverse effects*; D004298:Dopamine / Q000009:adverse effects*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D009638:Norepinephrine / Q000009:adverse effects*; D014662:Vasoconstrictor Agents / Q000009:adverse effects*
D002316:Cardiotonic Agents; D014662:Vasoconstrictor Agents; D001142:Aromatic-L-Amino-Acid Decarboxylases; D004298:Dopamine; D009638:Norepinephrine