Case Report


JAP  Volume 7 Issue 1


fentanyl 300mcg and propofol 100mg. Maintenance was achieved with proprofol and remifentanyl target-controlled infusion. Deep anaesthesia and haemodynamic stability was ensured prior to airway manipulation. Unfortunately we failed to place two different LMAs to our satisfaction so an endotracheal tube was placed via direct vision with a Mackintosh blade (Grade 2b/3) with assistance of a bougie and external laryngeal manipulation. We avoided local anaesthetic for both the vocal cords and for


nerve blocks due to the likely increased sensitivity to systemic toxicity in these patients.1,6 The patient was carefully positioned into the ‘beach-chair’ position in incremental steps whilst carefully maintaining haemodynamic stability. Attention was paid to maintain normovolaemia and normothermia. Intravenous paracetamol and diclofenac were administered and


a fentanyl patient-controlled-analgesia system was prepared for the post-operative period. The patient was extubated, breathing spontaneously under sedation, and awoke with power matching her pre-operative muscle strength. Although usually a day case procedure, she was precautionarily transferred to the high dependency unit for overnight monitoring of electrolytes, ECG and muscle power. Although it was not needed, we had planned a low dose propofol


infusion for problematic post-op nausea and vomiting, as we had to avoid ondansetron, dexamethasone3


and dopamine


antagonists (all QT prolonging). She was discharged home the following day.


Discussion ATS patients usually present in the first or second decade with


complex cardiac conditions and a variety of rhythm disturbances, commonly long QT syndrome, ventricular bigeminy, non-sustained VT and bidirectional ventricular tachy-dysrhythmia. Structural anomalies, such as bicuspid aortic valve, coarctation of the aorta and pulmonary valve stenosis, are often seen.1 During the surgical procedure there were no arrhythmias.


However, had a bradycardia occurred, it was planned for it to be treated by override pacing only as no atropine, glycopyrolate, adrenaline or derivatives should be used in these patients.3,4


a tachycardia occurred, it was planned for management with fluids, sedation, beta-blockers, defibrillation and/or Mg2+


/Ca2+


infusions as indicated. Amiodarone and Class I anti-arrhythmics are contraindicated and refractoriness to anti-arrhythmic agents is often a problem.6 The attacks of intermittent weakness or flaccid paralysis seen


in ATS occur either spontaneously or triggered by potassium administration, hypokalaemia, prolonged rest or exercise and potentially therefore, by the stress of anaesthesia. Both proximal


Dr W J Packer is an Anaesthesia Core Trainee at the University Hospital of Wales in Cardiff.


Dr M W M Strätling is a Consultant Anaesthetist, working at the same hospital.


Had


and distal muscles can be involved and some patients may have persistent generalised background weakness.1 Drugs that may have a beneficial effect on cardiac function may worsen skeletal muscle function (e.g. K+


/Mg2+ infusions), creating a


unique treatment challenge. In this case, intubation was optimised by remifentanyl infusion,


however, had neuromuscular blockade been required, we would have used rocuronium and then fully reversed with sugammadex. This is because the response to muscle relaxation is unknown in this patient group and also neostigmine and glycopyrolate are contraindicated.3 This lady’s elective procedure required significant research and planning, since there is little literature on Andersen-Tawil syndrome and the management of patients presenting with it, but ultimately this all paid off and resulted in an uneventful, safe anaesthetic. ■


JAP 2013: 1: 38-39


References 1. Tawil R, Venance S. Andersen-Tawil Syndrome. In: Pagon RA, Bird


TD, Dolan CR, Stephens K, Adam MP. Gene Reviews TM Internet edn. Seattle: University of Washington Press, 2010. http://www. ncbi.nlm.nih.gov/books/NBK1264/ (accessed 04/10/2012)


2. Airey KJ, Etheridge SP, Tawil R, Tristani-Firouzi M. Sudden cardiac death in Andersen-Tawil syndrome. Heart Rhythm 2009; 6: 1814-7


3. Centre for Education and Research on Therapeutics Arizona. Drugs that prolong the QT interval, 2012. http://www.qtdrugs. org/ (accessed 04/10/2012)


4. Sansone V, Tawil R. Management and treatment of Andersen- Tawil syndrome (ATS). Neurotherapeutics. 2007 Apr;4(2):233-7


5. Friederich P, Solth A, Schillemeit S, Isbrandt D. Local anaesthetic sensitivities of cloned HERG channels from human heart: comparison with HERG/MiRP1 and HERG/MiRP1T8A. British Journal of Anaesthesia 2004; 92 (1): 93-101.


6. Bendahhou S, Fournier E, Gallet S, Menard D,Larroque MM, Barhanin J. Corticosteroid-exacerbated symptoms in an Andersen’s syndrome kindred. Human Molecular Genetics 2007; (16)8:9000-6. Epub.


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