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Symptoms
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What to look for/be aware of
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Investigation & treatment and/or referral pathway
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Further reading
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Long QT
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An association between Rett Syndrome and Long QT has been noted, which may arise more commonly with increasing age.
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There are multiple meds which can increase the QT interval.
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Common drugs to avoid: Fluoxetine, Azithromycin.
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Entromycin with other meds can be lethal.
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Baseline ECG should be undertaken at diagnosis.
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ECG should be undertaken where there is suspicion of ECG abnormalities from clinical history and reported observational history.
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ECG should be undertaken where there is desire to prescribe medication which is known to potentially cause QT changes with 48 hour ECG recording obtained prior to medication change.
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Where negative, once treatment is prescribed, repeat ECG should be undertaken for seven consecutive days. (May be adjusted depending on rapidity of action of the drug).
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If positive for ECG changes and/or prolonged QT interval, drug should be withdrawn and ECG recording continued to ensure return to pre-treatment status.
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Where possible, avoid drugs which prolong QT interval, where there is alternative available.
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ECG should be conducted as part of pre-surgical assessment especially when anaesthetic is being given.
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Website list/smartphone app for drugs to avoid with Long QT
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Cardiovascular dysregulation
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Dysregulation of cardiovascular parameters. Heart rate, BP, peripheral vasoconstriction, may change markedly within seconds.
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Monitor for QTc prolongation at least annually.
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QTc may vary markedly within a short space of time.
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Beware of/ avoid medications causing QTc prolongation.
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Circulation
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Very poor flow to extremities especially lower leg/feet.
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Risk of severe chilblains.
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Doppler Scan.
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Thermal socks/footwear/foot spa.
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Warming slowly when cold, elevating feet.
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Consider Amlodipine for severe cases before considering GTN patches to decrease risk of severe headaches.
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Severe cases consider sympathectomy.
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Glyceryl trinitrate (GTN) patches may be helpful.
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