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Anaesthesia for children with  long QT syndrome Samia I. Sharaf , M.D Ain Shams University
Long QT Syndrome In Children  ( LQS) ,[object Object]
What Is QT Interval  ? How It Is Measured ? ,[object Object],[object Object],[object Object],[object Object],[object Object]
Classification Of LQTS ,[object Object],[object Object],[object Object],[object Object]
How QT Interval Is Prolonged ? ,[object Object],[object Object],[object Object],[object Object]
Congenital Long QT Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Congenital Long QT Syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object]
Genotypes Of Long QT Syndrome Ion channel affected  Chromosome/gene  Frequency  Phenotype  Genotype  K+(1K S ) 11-KVLQT1 +++ LQT1 LQT1 K+(1K R ) 7-HERG ++ LQT2 LQT2 NA+ 3-SCN5A + LQT3 LQT3 UNKNOWN UNKNOWN - LQT4 K+(1K S ) 21-KCNE1 - LQT1 LQT5 K+(1K R ) 21-KCNE2 - LQT2 LQT6
Clinical Picture Of c-LQTS ,[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical Picture Of c-LQTS ,[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical Outcome Of c-LQTS ,[object Object],[object Object],[object Object]
C-LQTS: Diagnosis, Schwartz Score ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
c-LQTS: Diagnosis, Schwartz Score ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Acquired LQTS Quinidine,procainamide,disopyramide,sotalol,amiodarone Antidysrhythmic drugs Terfenadine,astemizole Histamine receptor antagonists Erythromycin,clindamycin,imidazole,ampicillin Antibiotics  Ketanserin,flouxetine Drugs acting on serotonin receptors Tetra-and tricyclic antidepressant , haloperidol,phenothiazine,lithium,risperidone Antipsychotic drugs indapamide Diuretics  Massive blood transfusion  Citrate  Cisapride  Gastrointestinal drugs Hypokalemia , hypomagnesemia, hypocalcaemia Metabolic abnormalities  Complete atrioventricular block Bradydysrhythmias  Myocardial ischemis,acute carditis,acute cor pulmonale, mitral valve prolapse Cardiac  Anorexia nervosa,gastroplasty,liquid protein diet Starvation  Subarachinoid hge. Head injury,right-sided radial neck surgery Nervous system injury  Hypothermia, pituitary insufficiency Others
Treatment OF c-LQTS ,[object Object],[object Object],[object Object],[object Object],[object Object]
Treatment Of Torsade De Pointes Mechanism Of Torsade De Pointes ,[object Object],[object Object],[object Object],[object Object]
 
Treatment ,[object Object],[object Object],[object Object],[object Object]
Drugs Well Documented To Prolong  QT Interval Relevant To Anesthetic Practice ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Drugs That Prolong QT Interval In  Documented Case Reports ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Drugs To Be Avoided In Patients With  c-LQTS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Anesthesia For Patients With c-LQTS   General considerations  ,[object Object],[object Object],[object Object],[object Object],[object Object]
General Considerations For Conduct  Of Anesthesia In Patients With  c-LQTS ,[object Object],[object Object],[object Object],[object Object]
General Considerations For Conduct  Of Anesthesia In Patients With  c-LQTS ,[object Object],[object Object],[object Object]
Although information of the effect of some anaesthetic drugs on QT were available before significance of TDR of M cells yet the clinical significance is often unclear & it is difficult to advise with authority on which the safest anaesthetic agent for LQTS Anaesthetic Drugs & Ventricular  Repolarization
Premedication ,[object Object],[object Object],[object Object],[object Object],[object Object]
Intravenous Agents ,[object Object],[object Object],[object Object],[object Object]
Effects Of Sevoflurane Versus Propofol  On QT interval   Paventi S, Santevecchi A, Ranieri R.   ,[object Object],[object Object]
Methohexital : prolongs QTC in healthy adults but not in children ( despite atropine premedication )  Act. Anaesthesiol Scand, 1993 ketamine : unreported effect , better avoided Etomidate : has no significant effect  Act. Anaesthesiol Scand 1994
Volatile Anesthetics  Effects of Sevoflurane versus Propofol on QT interval .     Paventi S, Santevecchi A, Ranieri R.  ,[object Object],[object Object]
Volatile Anesthetics The effects of sevoflurane, isoflurane and desflurane on QT interval of the ECG. Yildirim H, Adanir T, Aday A, Katircioglu K. Savaci S .  ,[object Object],[object Object]
Volatile Anesthetics Anaesthesia and the QT interval in humans. The effects of isoflurane and halothane.   Michaloudis D, Fraidakis O, Lefaki T, Dede I, Kanakoudes F, Askitopoulou H, Pollard BJ. ,[object Object],[object Object]
Volatile Anesthetics Anaesthesia and the QT interval. Effects of isoflurane and halothane in unpremedicated children.   Michaloudis D, Fraidakis O, Petrou A. Gigourtsi C, Parthenakis F.  ,[object Object],[object Object]
[object Object]
Muscle Relaxants   The effects of midazolam followed by administration of either vecuronium or atracurium on the QT interval in humans.  Michaloudis DG, Kanakoudis FS, Xatzkraniotis A, Bischiniotis TS ,[object Object],[object Object]
Muscle Relaxants The effects of midazolam or propofol followed by suxamethonium on the QT interval in humans.   Michaloudis DG, Kanakoudis FS, Petrou AM, Konstantinidou AS, Pollard BJ.  ,[object Object],[object Object]
Muscle Relaxants ,[object Object],[object Object],[object Object]
Neuromuscular Reversal Effects of four anticholinesterse-anticholinergic combinations and tracheal extubation on QTc interval of the ECG, heart rate and arterial pressure.    Saarnivaara L, Simola M.  ,[object Object],[object Object]
Antiemetics Prolongation of QTc interval after postoperative nausea and vomiting treatment by droperidol or ondansetron .  Charbit B, Albaladerjo P, Funck-Brentano C, Legrand M, Samain E, Marty J  ,[object Object],[object Object]
Antiemetics Effect of low-dose droperidol on the QT interval during and after general anesthesia: a placebo controlled study    White PF, Song D, Abrao J, Klein KW, Navarette B. ,[object Object],[object Object]
Narcotics   ,[object Object],[object Object],[object Object],[object Object]
Conclusion   ,[object Object]

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Anesthesia for children with long QT syndrome

  • 1. Anaesthesia for children with long QT syndrome Samia I. Sharaf , M.D Ain Shams University
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  • 8. Genotypes Of Long QT Syndrome Ion channel affected Chromosome/gene Frequency Phenotype Genotype K+(1K S ) 11-KVLQT1 +++ LQT1 LQT1 K+(1K R ) 7-HERG ++ LQT2 LQT2 NA+ 3-SCN5A + LQT3 LQT3 UNKNOWN UNKNOWN - LQT4 K+(1K S ) 21-KCNE1 - LQT1 LQT5 K+(1K R ) 21-KCNE2 - LQT2 LQT6
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  • 14. Acquired LQTS Quinidine,procainamide,disopyramide,sotalol,amiodarone Antidysrhythmic drugs Terfenadine,astemizole Histamine receptor antagonists Erythromycin,clindamycin,imidazole,ampicillin Antibiotics Ketanserin,flouxetine Drugs acting on serotonin receptors Tetra-and tricyclic antidepressant , haloperidol,phenothiazine,lithium,risperidone Antipsychotic drugs indapamide Diuretics Massive blood transfusion Citrate Cisapride Gastrointestinal drugs Hypokalemia , hypomagnesemia, hypocalcaemia Metabolic abnormalities Complete atrioventricular block Bradydysrhythmias Myocardial ischemis,acute carditis,acute cor pulmonale, mitral valve prolapse Cardiac Anorexia nervosa,gastroplasty,liquid protein diet Starvation Subarachinoid hge. Head injury,right-sided radial neck surgery Nervous system injury Hypothermia, pituitary insufficiency Others
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  • 23.
  • 24.
  • 25. Although information of the effect of some anaesthetic drugs on QT were available before significance of TDR of M cells yet the clinical significance is often unclear & it is difficult to advise with authority on which the safest anaesthetic agent for LQTS Anaesthetic Drugs & Ventricular Repolarization
  • 26.
  • 27.
  • 28.
  • 29. Methohexital : prolongs QTC in healthy adults but not in children ( despite atropine premedication ) Act. Anaesthesiol Scand, 1993 ketamine : unreported effect , better avoided Etomidate : has no significant effect Act. Anaesthesiol Scand 1994
  • 30.
  • 31.
  • 32.
  • 33.
  • 34.
  • 35.
  • 36.
  • 37.
  • 38.
  • 39.
  • 40.
  • 41.
  • 42.