[Congenital long QT syndrome in newborns].
The perinatal manifestations of the long QT syndrome are rare, but early diagnosis and therapy are necessary to prevent sudden death.


METHODS
A long QT syndrome was diagnosed in two neonates who presented with foetal bradycardia. In one case, a mutation in the gene KCNQ1 was identified, and a long QT syndrome was diagnosed in the mother and two brothers of the neonate. On beta-blocker therapy, one infant became free of long QT syndrome related symptoms, but a sudden death of the second infant occurred.


CONCLUSIONS
The long QT syndrome should be considered in the differential diagnosis of foetal bradycardia. Early treatment of the neonate and his family may prevent ventricular arrhythmias and sudden death.
Emeriaud
G
G
Service de réanimation néonatale et infantile, CHU de Grenoble, BP 217, 38043 Grenoble, France. guilemeriaud@hotmail.com
Douchin
S
S
Jouk
P S
PS
Andrini
P
P
Wroblewski
I
I
Marey
C
C
Rossignol
A M
AM
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D001919:Bradycardia / Q000209:etiology*; D004252:DNA Mutational Analysis; D016757:Death, Sudden, Cardiac; D003937:Diagnosis, Differential; D004562:Electrocardiography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; D008133:Long QT Syndrome / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy; D010375:Pedigree; D015221:Potassium Channels / Q000235:genetics; D024642:Potassium Channels, Voltage-Gated
D000319:Adrenergic beta-Antagonists; D051656:KCNQ Potassium Channels; D051657:KCNQ1 Potassium Channel; C496909:KCNQ1 protein, human; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated