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Arch Mal Coeur Vaiss. 2004 May;97(5):479-83.

[Neonatal forms of congenital long QT syndrome].

[Article in French]

Author information

1
Cardiologie pédiatrique, hôpital Robert Debré, 48, boulevard Sérurier, 75019 Paris. jean-marc.lupoglazof@rdb.ap-hop-paris.fr

Abstract

The neonatal congenital long QT syndrome (LQTS) is rare and of bad prognosis due to the presence of severe ventricular arrhythmia and conduction abnormalities.

METHODS:

we included 24 propositus newborns from our population with LQTS. Genetic study was possible in 19 cases.

RESULTS:

the diagnosis of LQTS was made according to a QT prolongation associated with a sinusal neonatal bradycardia (n=9) or a 2/1 AV block (n=15). The onset presentation consisted of syncope (n=2), torsades de pointes (n=7), cardiovascular collapse (n=5), cardiac arrest (n=1). The mean QTc was at 550+60 ms. During the neonatal period the treatment consisted of beta-blocking agents in all cases, associated with a definitive pacemaker implantation in 10 cases with 2/1 AV block. Three newborns with a 2/1 AV block died during the first month of life (one case due to a septecemia after implantation of a pacemaker, and two who were waiting for that implantation). All survivors remained asymptomatic during a follow-up period of 7 years. In all cases with a 2/1 AV block we identified mutations in HERG (n=8). Newborns with isolated sinusal bradycardia presented all a mutation in KCNQ1 (n=9).

CONCLUSION:

the LTQS with 2/1 AV block is preferably associated with mutation in HERO with a bad initial prognosis.

PMID:
15214551
[Indexed for MEDLINE]

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