LQTS


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LQTS

Long QT interval syndrome, see there.
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This includes a comprehensive clinical and genetic evaluation; a consultation with LQTS experts as needed; extensive counseling regarding individual risks and athletic guidelines; tailored therapy (beta-blockers, left cardiac sympathetic denervation, and/or an ICD); education regarding appropriate hydration and electrolyte replenishment; minimizing elevations of core body temperature; and avoidance of QT drugs, the authors noted.
Published guidelines direct the management of patients with LQTS to reduce the risk of sudden cardiac death (10).
Ninety percent of the known mutations cause loss of function of ion channels responsible for LQTS types 1 and 2 (LQT1 and LQT2).
This patient had baseline sinus node dysfunction and underlying LQTS.
Romano-Ward syndrome is the most common inherited form of LQTS and is transmitted as an autosomal-dominant trait[1,2.
The girls and Mary Ann - who has had the disorder since birth and had fainting spells when she was younger - now take beta blockers, the mainstay therapy that is effective in about 90 percent of LQTS subjects.
The prevalence of LQTS is estimated to be 1 in 2,000-5,000; however, the expressivity, or penetrance, of the syndrome is highly variable even within the same family.
As a consequence, under baseline conditions, mutations in genes controlling normal repolarization may remain subclinical and may only be the source of clinical and electrocardiographic manifestations of the LQTS upon exposure to further stressors, such as drugs or bradycardia.
These findings from a unique database housed at LDS Hospital are at odds with an earlier report by other investigators, who reported that the postpartum period in women with LQTS was associated with a 41-fold increased rate of cardiac events (Circulation 1998;97:451-6).
A known family history of hypertrophic cardiomyopathy, LQTS, cardiomyopathy or ARVD warrants further work-up.