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18 Cards in this Set

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What is long QT syndrome?
It is an electrical disease of the ventricular myocardium.
What is QT syndrome characterized by?
By prolonged ventricular repolarization, which results in the prolongation of the Q-T interval on the surface ECG and an increased risk of sudden death.
Is long QT syndrome associated with any other type of arrhythmias?
Yes. Torsade de pointe, which is a form of polymorphic ventricular tachycardia.
Is QT syndrome acquired?
It can be. It can also be congenital.
What causes congenital QT syndrome?
Possibly by mutations in the gene coding for the cardiac K+, Na+ or calcium ion channels.
Are there distinct genetic types?
Yes.They are designated, LQT1-LQT10.
Types 1,2 and 3 account for over 90% of cases.
What is Romano-Ward syndrome?
It is inherited in an autosomal dominant fashion and only has cardiac manifestations.
What is Jervell and Lange-Nielsen syndrome?
Also inherited, but in an autosomal recessive fashion and is associated with sensorineural deafness.
What is the app. incidence of LQTS?
About one case in 2500-10,000. Perhaps many cases go undiagnosed
About 20-50% of affected patients may not demonstrate QT prolongation on a resting EKG. TRUE/FALSE
TRUE. It is one of the most common causes of autopsy negative unexplained sudden death.
Women are more often affected than men. TRUE/FALSE
TRUE.
How do early cases present?
Palpitations, syncope or near syncope, seizures or cardiac arrest.
Syncopal episodes associated with 2ary seizures may be misdiagnosed as primary seizure disorders. TRUE/FALSE
TRUE. Seizures are likely secondary to hypoperfusion of the brain during arrhythmic events.
Cardiac arrhythmias can be triggered by what?
An external trigger, emotional stress, exercise, or sudden loud noises. Mortality can be as high as 70% in patients who remain untreated over a 10 year period.
What are some treatment guidelines employed?
Lifestyle modifications, defined as the contraindication of competitive sports and of all drugs known to prolong the QT interval.
What is the mainstay of treatment?
Use of beta blockers. Beta blockers shorten the QT interval which diminishes the risk of torsade de pointe. These are effective in app. 70% of patients.
What can also be done in high risk patients?
Implantable cardioverter defibrillators(ICDs) appear to be effective.
What is mexiletine?
It is a sodium channel blocker and has been demonstartaed to shorten the QT interval in the subgroup of patients with LQT3.