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Accepted for publication 22nd November, 1990.The Jervell and Lange-Nielsen syndrome (JLNS) is characterized by congenital deafness, long QTc interval and microcytic hypochromic anaemia. It is an unusual familial cause of syncopal attacks and sudden death during infancy and childhood. ~ It is now recognised that the syncopal attacks in these patients are due to polymorphous ventricular tachycardia. 2'3 However, these recurrent syncopal attacks in children with JLNS may be misdiagnosed as epilepsy 4-6 and general anaesthesia may trigger a potentially fatal arrhythmia. 7-9We report a child, previously diagnosed as epileptic, in whom halothane anaesthesia induced polymorphous ventricular tachycardia. This led to the diagnosis of JLNS.Case report A four-year-old girl with a history of recurrent epileptiform attacks was scheduled for brain scan under general anaesthesia on an out-patient basis. There was no previous history of anaesthesia. She was deaf and showed no other neurological deficit. Apart from mild microcytic hypochromic anaemia (Hb 9.2 g. d1-1, Hct 29.7%, MCH 19.5 pg-dl -l, MCHC 31%, MCV 62, anisocytosis), all other laboratory investigations were normal. No outpatient pre-anaesthetic ECG was done. Pre-induction EGG appeared normal. An iv infusion of dextrose 0.5% was started and atropine 0.2 mg given iv. There was a moderate sinus tachycardia. Anaesthesia was induced with nitrous oxide in 50% oxygen and halothane given in incremental concentrations up to 4%. At laryngoscopy, and prior to insertion of the endotracheal tube, the ECG showed polymorphous ventricular tachycardia ( Figure lA) which spontaneously reverted to sinus rhythm. A size 4.5 mm endotracheal tube was inserted and anaesthesia was maintained with 1% halothane in 100% oxygen. No convulsion occurred during anaesthesia. The CAT scan revealed no brain pathology. The patient was transferred to the paediatric cardiology clinic where a diagnosis of Jervell and Lange-Nielsen syndrome was made on the basis of clinical features and ECG characteristics ( Figure IB).
Accepted for publication 22nd November, 1990.The Jervell and Lange-Nielsen syndrome (JLNS) is characterized by congenital deafness, long QTc interval and microcytic hypochromic anaemia. It is an unusual familial cause of syncopal attacks and sudden death during infancy and childhood. ~ It is now recognised that the syncopal attacks in these patients are due to polymorphous ventricular tachycardia. 2'3 However, these recurrent syncopal attacks in children with JLNS may be misdiagnosed as epilepsy 4-6 and general anaesthesia may trigger a potentially fatal arrhythmia. 7-9We report a child, previously diagnosed as epileptic, in whom halothane anaesthesia induced polymorphous ventricular tachycardia. This led to the diagnosis of JLNS.Case report A four-year-old girl with a history of recurrent epileptiform attacks was scheduled for brain scan under general anaesthesia on an out-patient basis. There was no previous history of anaesthesia. She was deaf and showed no other neurological deficit. Apart from mild microcytic hypochromic anaemia (Hb 9.2 g. d1-1, Hct 29.7%, MCH 19.5 pg-dl -l, MCHC 31%, MCV 62, anisocytosis), all other laboratory investigations were normal. No outpatient pre-anaesthetic ECG was done. Pre-induction EGG appeared normal. An iv infusion of dextrose 0.5% was started and atropine 0.2 mg given iv. There was a moderate sinus tachycardia. Anaesthesia was induced with nitrous oxide in 50% oxygen and halothane given in incremental concentrations up to 4%. At laryngoscopy, and prior to insertion of the endotracheal tube, the ECG showed polymorphous ventricular tachycardia ( Figure lA) which spontaneously reverted to sinus rhythm. A size 4.5 mm endotracheal tube was inserted and anaesthesia was maintained with 1% halothane in 100% oxygen. No convulsion occurred during anaesthesia. The CAT scan revealed no brain pathology. The patient was transferred to the paediatric cardiology clinic where a diagnosis of Jervell and Lange-Nielsen syndrome was made on the basis of clinical features and ECG characteristics ( Figure IB).
A case report of a patient known to have long Q-T syndrome (LQTS) undergoing elective oral surgery is presented. While nitrous oxide-narcotic, nitrous oxideenflurane, or nitrous oxide-halothane techniques for anaesthetic management of LQTS have been previously reported, we report the use of nitrous o.tide-isoflurane for the maintenance of anaesthesia. The authors feel that isoflurane is a safe anaesthetic agent for use in LQTS. Key wordsHEART: long Q-T syndrome, electrocardiography; ANAESTHETICS, VOLATILE: isoflurane; SURGERY: oral.Congenital long Q-T syndrome (LQTS) with syncopal episodes secondary, to ventricular tachyarrhythmias following emotional or physical stress has been described, l Ventricular tachyarrhythmias typically follow stimulation of the sympathetic nervous system secondary to stress, including the stress of anaesthesia and surgery. Indeed, unrecognized or improperly managed congenital LQTS may lead to intraoperative ventrieular tachyarrhythmias and even death?Although extensive medical literature on LQTS exists,~ descriptions of anaesthetic management are limited. Review of the literature indicates there have been only seven patients reported with this syndrome who have received anaesthesia? -7 These patients have been managed with nitrous oxideenflurane, nitrous oxide-halothane, and nitrous oxide-narcotic techniques. The following case is, to our knowledge, the first report of a palien! with LQTS anaesthetized with isoflurane. Case reportA twenty-seven-year-old woman was scheduled for elective mandibular osteotomy. The diagnosis of LQTS was made three years prior to admission after the patient presented with a history of syncopal episodes since childhood. The patient had been well controlled on propranolol and diphenylhydantoin for three years. Three family members had LQTS with one death from the syndrome. There was no personal or family history of deafness. Electrocardiograms prior to admission documented long Q-T intervals with episodes of ventficular tachycardin. The patient's history was otherwise unremarkable. Physical examination on admission revealed a well-developed, well-nourished, white female in no acute distress. Heart rate was regular at 60 beats per minute with a blood pressure of !30/85 tort, respiratory rate was 16 per minute, and temperature was 36,7 ~ C. The patient was 5'8" tall and weighed 57 Kg. Examination of the heart, chest, abdomen, and CNS were within normal limits. Haemoglobin (hgb), haematocrit, blood urea nitrogen, serum creatinine, sodium (No), potassium (K), and calcium were within the normal range on admission. A standard 12 lead electrocardiogram (see Figure) showed a sinus bradycardia with a rate of 60 beats per minute. The Q-T interval was 0.44 s and there were prominent U waves.Hydromnrphone hydrochloride 0.5 mg, hydroxyzine hydrochloride 50mg, and glycopyrrolate 0.2 mg intramuscularly were given two hours prior to surgery. On arrival to the preoperative holding area, we instituted continuous electrocardiographic monitoring. Diazepam 10 mg and morphine sul...
P Pu ur rp po os se e: : Congenital long QT syndrome is characterized by a corrected QT interval of at least 440 msec on the electrocardiogram and has been associated with recurrent syncope, documented ventricular arrhythmia and sudden death. There have been numerous articles over the past 20 years describing isolated instances of surgical and anesthesia related complications but the general anesthetic management of the condition remains unclear.C Cl li in ni ic ca al l f fe ea at tu ur re es s: : An 11-yr-old female with documented long QT syndrome, with two episodes of syncope in the past, was admitted for emergency drainage of left periorbital cellulitis. Anesthesia was induced with propofol, fentanyl and rocuronium, and initially maintained with nitrous oxide and halothane. After 20 min, the patient developed ventricular tachycardia (torsade de pointes). Lidocaine 1 mg·kg -1 iv was given and the rhythm reverted to normal sinus. Halothane was discontinued and the surgery proceeded without further incident.C Co on nc cl lu us si io on ns s: : Our review of the literature revealed that patients with long QT syndrome whose symptoms are well controlled prior to surgery tend to do well regardless of the anesthetic chosen. There are, however, theoretical reasons to avoid anesthetics which either sensitize the myocardium to catecholamines or which cause an increase in circulating levels of catecholamines.
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