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Long QT Syndrome
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  • Long QT Syndrome (LQTS):Symptoms,Causes,Diagnosis and Treatment

    Long QT syndrome (LQTS) results from structural abnormalities in the potassium channels of the heart, which predispose affected persons to an accelerated heart rhythm (arrhythmia). This can lead to sudden loss of consciousness and may cause sudden cardiac death in teenagers and young adults who are faced with stressors ranging from exercise to loud sounds.

    http://heart-consult.com/articles/long-qt-syndrome-lqtssymptomscausesdiagnosis-and-treatment
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  • 1. ECG of the week
    • Dr. DevendraPatil
    Prof.Dr. S.Magheshkumar’sUnit
  • 2. Indira 70/ F Retd. Matron GSH came with chief
    complains of
    Fever since 20-25 days
    Intermittent ,high grade asso. with chills n rigors
    Taking some oral medication from pvt.
    K/c/o SHT and DM since 15 yrs on Rx.
    2 days later she had a cardiac arrest with the following ECG pattern
  • 3.
  • 4.
  • 5. ECG findings :-
    Heart Rate – 50 bpm
    Regular Sinus rhythm
    Normal Axis
    P wave - normal
    PR interval - 0.12 sec
    QRS complex – normal width and shape
    QT interval – 0.56 sec
    QTc interval - 0.509 sec
    T wave – normal
    Impression :- Bradycardia with acquired prolonged QT syndrome
    Pt. was shifted to ICCU.
  • 6. She developed cardiac arrest .
    Pulse – not felt
    BP - not recordable
    ECG showed Vent. Tachycardia
    200 J Biphasic DC shock was given and was reverted to sinus rhythm
  • 7. A Temporary Cardiac Pacemaker was inserted following Rt. Femoral Vein Catheterization and she was reverted to sinus rhythm over the next 2 days
  • 8. QT interval : beginning of Q-wave upto the end of T-wave.
    QT interval : total duration of ventricular activity.
    Need for QTc Interval :
    The QT interval varies with RR interval. False high values may be noted in Bradycardias.
    LONG QT SYNDROME
  • 9. Formula accepted for QTc is:
    BAZETT’s Formula : QTc = QT ∕√ (RR)
    A useful rule of thumb is that, with a normal heart rate ( 60 - 100 bpm), the QT interval SHOULD NOT exceed half the RR interval.
  • 10. LONG QT SYNDROME
    CONGENITAL
    ACQUIRED
    Due to Genetic defects in K+ channels involved during repolarization.
    Severe form Includes:
    1. Jervell Lange Syndrome
    (deafness, syncopal attacks , sudden death )
    2. Romano Ward Syndrome (similar to Jervell Lange syndrome but without deafness )
  • Congenital LONG QT SYNDROME
    LQT1 Gene
    Most common type
    QT interval fails to shorten post exercise
    T wave is broad and occupies majority QT interval
    Most common triggers: exercise, emotional stress.
    80% have first presentation < 20 yrs
    Jervell Lange syndrome and Romano Ward syndrome are severe forms.
    LQT2 Gene
    T wave tends to be bifid and notched
    Additional Triger factors are sleep, auditory stimulation
    Respond to B-Blockers
    LQT3 Gene
    Poorest prognosis amongst all
    T wave is asymmetrical or Late biphasic
    Trigger factor : Bradycardia ( sleep )
    B-Blockers Contraindicated
    Exercise is not Restricted
  • 19. DRUGS that may cause QT PROLONGATION
    AntiarrhythmicsClass 1: ajmaline*, cibenzoline*, dihydroquinidine*, disopyramide, encainide*, flecainide, mexiletine, pirmenol*, procainamide,propafenonequinidine*Class 3:almokalant*, amiodarone, azimilide*, bretylium, dofetilide*, dronedarone*, d-sotalol*, ersentilide*, ibutilide*, nifekalant*, sematilide*, sotalol, terikalant*
    Anti-anginals/vasodilatorsbepridil*, lidoflazine*, prenylamine*, ranolazine, terodiline*, vardenafil
    Anti-hypertensivesindapamide, isradipine, moexipril/hydrochlorthiazide, nicardipine
    Antihistaminesastemizole*, azelastine, diphenhydramine, ebastine*, hydroxyzine, terfenadine*
    Serotonin agonists and antagonistscisapride*, dolasetron, granisetron, ketanserin*, ondansetron
    AntimicrobialsMacrolide antibiotics: azithromycin, clarithromycin, erythromycin, roxithromycin*, spiramycin, telithromycin
    Quinolone antibiotics: ciprofloxacin, gatifloxacin*, gemifloxacin*, grepafloxacin*, levofloxacin, moxifloxacin, ofloxacin, sparfloxacin*
    Antifungals: cotrimoxazole, fluconazoleOthers: pentamidine, trimethoprimsulfaAntiviral: foscarnet (HIV)
  • 20. DRUGS that may cause QT PROLONGATION
    Continued…
    AntimalarialsChloroquine, halofantrine*, lumefantrine, quinine
    Psychiatric drugsTricyclic antidepressants: amitriptyline, amoxapine*, clomipramine, desipramine*, doxepin, imipramine, nortriptyline, protriptyline*, trimipramine
    Phenothiazines: chlorpromazine, fluphenazine, prochlorperazine, thioridazine*, trifluoperazine
    Others: atomoxetine, citalopram, clozapine, droperidol*, fluoxetine, haloperidol, levomethadyl*, lithium, maprotiline, mesoridazine, methadone, paroxetine, pericycline, pimozide, quetiapine, risperidone, sertindole, sertraline, trazodone, venlafaxine, zimeldine*, ziprasidone
    Anticonvulsantfelbamate*, fosphenytoin
    Anti-migrainenaratriptan, sumatriptan, zolmitriptan
    Anti-cancerarsenic trioxide, geldanamycin*, sunitib, tacrolimus, tamoxifen
    Othersalfuzosin,chloral hydrate, clobutinol*, domperidone, galantamine, octreotide, organophosphates*, perflutren lipid microspheres, probucol, solifenacin, tizanidine, tolterodine, vasopressin
    Stimulant drugsSome cold remedies contain these drugs so it is important always to check the label.adrenaline (epinephrine), amphetamine, cocaine, dexmethylphenidate, dobutamine, dopamine, ephedrine, fenfluramine, isoprenaline (isoproterenol), levalbuterol, metaproterenol, methylphenidate, midodrine, norepinephrine (noradrenaline), phentermine, phenylephrine, phenylpropanolamine, pseudoephidrine, ritodrine, salbutamol (albuterol), salmeterol, sibutramine, terbutaline
  • 21. Complications OF Long qt syndrome
    Torsades des pointes ( polymorphic ventricular tachycardia )
    More commonly seen in women
    Manifestation of subclinical LQTS
    Potentiated by bradycardia and hypokalemia
    drug usually blocks Ikr channel
    triggered by early afterdepolarizations due to intracellular Ca+ accumulation from a prolonged actin potential plateau
    heterogenicity of myocardial repolarisation predisposes to polymorphic ventricular activity
  • 22. Treatment
    Caution has to be exercised during administering multiple drug regimes. They may cause QT Prolongation and TDP.
    Correction of K+ and Mg++ levels
    Temporary Pacing
    Cautious Infusion of Isoprotenol
    Class 1B anti arrythmics(no QT prolongation seen)
  • 23. THANK YOU