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Verlengd qt syndroom

VERLENGD QT SYNDROOM

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1. Oorzaken

- mechanismen
- wijzigingen in cardiaal intracellulair natrium, kalium of calcium.  Hierdoor ontstaaty een verlengde ventrikelrepolarisatie, wat zich op ECG uit in een verlengd QT-interval en ritmevariaties (sinusaal complex, een extrasystole, daarna een pauze).  Dit geeft risico op reëntry tachycardie met torsades de pointes, ventrikeltachycardie of ventrikelfibrillatie.
- toxicologishe oorzaken:
- vaak is er een genetische achtergrond.
- medicatie:
- anti-arhytmica
- klasse 1a (quinidine,  procainamide, disopyramide)
- klasse 3 (sotalol, ibutilide, amiodarone)
- antibiotica
- erythromycine
- pentamidine
- chloroquine
- trimethoprim-sulfamethoxazole
- antihistaminica
- psychotrope medicatie
- phenothiazines
- haloperidol
- risperidone
- TCA's
- insecticiden: organofosfaten
- cardiale oorzaken
- bradyarrhytmieën
- AV blok
- mitralisklepprolaps
- myocarditis
- myocardischaemie
- metabole oorzaken
- electrolietenstoornissen: hypokaliëmie, -magnesiëmie of -calciëmie
- hypothermie
- hypothyroidie
- dieet (proteinearm, anorexia nervosa)
- centrale zenuwstelsel - oorzaken
- CVA
- subarachnoidale bloedin
- congenitale vorm, soms samengaand met congenitale doofheid

- voorkomen bij kinderen: mogelijkheid bij syncopes na stress of inspanning, hartstilstand of plotse dood.  Bij 10% is er een overlijden zonder eerdere symptomen.

 

2. Diagnose

- anamnese:
- syncopes:
- in het verleden?  ritmestoornissen? aanvallen van tachycardie?
- familiale gevallen van plotse dood
- ijlhoofdigheid
- stuipen
- genetische belasting
- congenitale doofheid
- familiale syncopes en plotse dood
- medicatiegebruik
- anti-artimica
- antibiotica
- antihistaminica
- psychotropica
- contact met insecticides
- electrocardiogram
- QT-interval: > 0,44s (> 11 kleine vakjes), variabel interval
- breed complex tachycardie (VT, VF of torsades de pointes)
- T-golf afwijkingen: T-golf alterans, bifasische T
- U-golven
- labo
- electrolieten
- toxicoscreening
- genetisch onderzoek
- echocardio
- uitsluiten andere oorzaken
- ECG stress testen: verlengen van QT-interval
- holtermonitoring

- familiale ECG-testen

 

3. DD

- acuut myocardinfarct
- kleplijden
- hypertrofische cardiomyopathie

 

 

Eerste opvang

- IV lijn
- monitoring
- is de patient stabiel?
- zo neen: cardioversie ter plaatse
- zo ja: met torsades de pointes?
- indien neen :gewoon transport
- indien ja: magnesiumsulfaat.
- in bolus 2 gram over 2 minuten.  (kind: 25-50mg/kg)

- daarna continu IV 2-4 mg/min

 

Spoeddienst

- indien torsades de pointes
- magnesiumsulfaat 2 gr IV over 2 min in bolus (kind 25-50 mg/kg).  Daarna 2-4 mg/min in continu IV infuus
- tijdelijke transveneuse pacing tussen 90 en 110/min
- indien pacing niet mogelijk is, eventueel Isuprel bij recidieven en bij instabiele patienten.
- correctie van electrolietenstoornissen
- stop evtl uitlokkende medicatie
- opname cardiologie voor
- monitoring
- tijdelijke transveneuse pacing tussen 90 - 110/min
- plaatsen van definitieve pacemaker en/of defibrillator

- opstarten hoge dosissen betablokkers ter preventie van ritmestoornissen

 

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