Perinatal manifestations of idiopathic long QT syndrome

Abstract

A neonate who had presented with sustained irregular heart rate during labor was found to have QT prolongation and repetitive polymorphic ventricular tachycardia (torsades de pointes) postnatally. Propranolol and propafenone successfully controlled the ventricular arrhythmias. Follow-up electrocardiograms and Holter records show persistent QT prolongation, bizarre T waves, and intermittent episodes of T wave alternans. On propranolol monotherapy the boy is thriving and completely free of ventricular arrhythmias. In the rare case of long QT syndrome in the neonate, early detection and therapy are mandatory to prevent ventricular arrhythmias and sudden death.

Impact of Sleep on Arrhythmogenesis

“…The genetic locus of the defect and the length of the QT interval are independent predictors of risk, suggesting opportunities for assessing risk for SIDS through molecular screening as well as noninvasive ECG monitoring 77,78. T-wave alternans has been reported in infants who became SIDS victims79 or were successfully treated 80…”

“…Prospective studies are required to examine the potential of sodium channel blockade or cardiac pacing in treating infants diagnosed with the long QT3 syndrome 78. Beta-blockade is the current treatment of choice76,80 and diminishes T-wave alternans magnitude 80…”

Fetus with long QT syndrome manifested by tachyarrhythmia: a case report

“…Ventricular tachycardia may precede the syncopal attack (Benhorin and Medina, 1997) and sudden death in these patients. Although cardiac arrhythmia has been detected in a small number of fetuses with LQTS, most of these cases were associated with fetal bradycardia (Southall et al, 1979;Bharati et al, 1985;Presbitero et al, 1989;Vigliani, 1995;Trippel et al, 1995;Mache et al, 1996). We describe a neonate who presented with tachyarrhythmia in utero and in whom LQTS was diagnosed postnatally.…”

Fetal and Neonatal Presentation of Long QT Syndrome

“…LQTS has been described in neonates, 2 but there have been few case reports of fetal presentation of LQTS 3,4 . Perinatal presentation or recognition of LQTS is rare, especially in the absence of a positive family history 5 . The disease is genetically heterogeneous and is caused by mutations in one of several genes including KCNQ1 , KCNH2 , KCNE1 , and KCNE2 encoding potassium‐channel subunits, the cardiac sodium‐channel gene SCN5A , and the L‐type calcium‐channel gene CACNA1C 6 .…”