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ANAESTHESIA FOR CAESAREAN SECTION AND THE JERVELL LANGENIELSON SYNDROME (PROLONGED Q-T INTERVAL SYNDROME)
Br. J. Anaesth. (1984), 56,655
ANAESTHESIA FOR CAESAREAN SECTION AND THE JERVELL, LANGENIELSON SYNDROME (PROLONGED Q-T INTERVAL SYNDROME) J. V. FRESHWATER SUMMARY
The syndrome of congenital deafness, syncopal attacks and an abnormal ECG showing prolongation of the Q - T interval corrected for heart rate (Q-Tc > 0.45 s) was described first by Jervell and LangeNielson (1957). It is a rare hereditary disease, inheritance being of the autosomal recessive type. The ECG abnormality resembles closely that found in the Romano-Ward syndrome which, however, is not associated with deafness, and has probably an autosomal dominant mode of inheritance. Both may present with a family history of syncope and of sudden death, often in infancy, occurring after exercise or emotional stress. Syncope may occur as a result of Stokes-Adams attacks, asystole or ventricular fibrillation occurring in the presence of atrio-ventricular block. Patients with prolonged Q - T interval syndromes may exhibit the "Torsades des pointes" phenomenon, polymorphic ventricular tachycardia, a rapid ventricular tachycardia characterized by changing QRS morphology. This is often self-terminating, but may degenerate into established ventricular fibrillation. Recent advances in therapy, notably the use of 0-adrenoceptor blocking drugs, have improved survival, and a few patients presenting for surgery and anaesthesia are now recorded in the literature. The patient described is one of the few to have the classic Jervell, Lange-Nielson (JLN) syndrome and, possibly, the first reported to undergo Caesarean section. CASE REPORT
A 20-year-old deaf Sikh female known to have the JLN syndrome presented for the management of her pregnancy. From age Syr she was subject to JANE V. FRESHWATER, M.B., CH.B., F.F.A.R.C.S., Department of
Anaesthetics, Western General Hospital, Crewe Road South, Edinburgh. Present address: Mooklands District General Hospital, Monkscourt Avenue, Airdrie, Lanarkshire.
Stokes-Adams syncopal attacks most frequent during her early childhood. Two siblings had died in infancy. At 9yr the JLN syndrome was diagnosed (Q-Tc 0.53 8) and treatment initiated with digoxin 0.062 5 mg daily. This was later changed to practolol 200 mg twice a day with good control of the attacks. Propranolol 20 mg twice per day was substituted later. On admission to hospital the patient had had no attacks for 3 years in spite of infrequent attendance for follow-up and, probably, poor compliance with therapy. She had an uneventful pregnancy, receiving propranolol 20 mg twice per day and was booked for elective Caesarean section under general anaesthesia. She was admitted for full preoperative assessment, including ECG. Propranolol 20 mg twice per day was continued until the morning of her operation. She was premedicated with diazepam 10 mg by mouth and after pre-oxygenation, cricoid pressure was applied. Anaesthesia was induced with thiopentone 250 mg, and suxamethonium 70 mg given to facilitate tracheal intubation. Ventilation was controlled, and anaesthesia maintain^ with nitrous oxide and 0.5% halothane in oxygen. She received intermittent injections of suxamethonium 50 mg during the 40-min procedure. A live, healthy male infant was delivered which was found to have no ECG abnormality. Immediately following operation the mother received papaveretum 5mg, cyciizine 25mgi.m. and diazepam 2.5 mgi.v. She had shown no additional ECG abnormalities throughout the procedure, and her course after operation was uneventful. Two years later the patient presented for the management of her second pregnancy, and requested sterilization. She had remained wellcontrolled on propranolol 20 mg twice per day, with no further syncopal attacks. She was prepared for general anaesthesia as before, receiving her morning © The Mtrmillan Press Ltd 1984
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
The anaesthetic management is described of a patient with Jervell, Lange-Nielson OLN) syndrome presenting on two occasions for Caesarean section. The syndrome, its treatment and the choice of anaesthesia are discussed.
656
BRITISH JOURNAL OF ANAESTHESIA
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
Propranolol is the treatment of choice. Its beneficial effect is probably not a result of its influence on the Q-T interval, which it tends to prolong, but rather to direct anti-sympathetic activity, and an ability to increase the threshold for ventricular fibrillation (Milne, Camm and Ward, 1980). Unfortunately, not all patients tolerate therapy or are controlled adequately, and further anti-arrhythmic management may be difficult. LJgnocaine and phenytoin have been used following surgery (Brown, et al., 1981). Percutaneous left stellate ganglion block has proved useful (Ponte and Lund, 1981), and surgical sympathectomy could be offered for long-term management. The choice of anaesthetic technique for these patients is difficult. Anaesthesia must be hazardous where there is a risk of a life-threatening arrhythmia. The choice in this patient was further limited by her pregnancy since any technique chosen had to DISCUSSION allay anxiety, provide cardiovascular stability, and JLN syndrome is an autosomal recessive condition. satisfy the strict criteria for safety of mother and Heterozygotes are clinically normal although their child inherent in any obstetric anaesthetic. Communication between the patient and the ECG may show prolongation of the Q-T interval. The frequency in the general population is 1 in medical team was restricted by her severe deafness 300 000 and in children with perceptive deafness, 1 and language problems. This difficulty and the risks in 100. Consanguinity of parents predisposes to the of possible hypotension in the presence of 0syndrome (Matthews, Blaint and Townsend, 1972): blockade excluded extradural anaesthesia as a suitaSikhs usually inter-marry within their own com- ble technique. General anaesthesia, using a well munity. The frequency of the syncopal attacks di- established technique and careful monitoring, minishes with age (Ratshin, Hunt and Russell, offered the safest choice. 1971). Syncope in patients with prolongation of the Preoperative visits, premedicants, the presence of Q - T interval is usually related to exercise; in this a familiar relative in the induction room, and the patient emotional stress was a precipitating factor. excellent rapport between patient and cardiologist Exercise tolerance was not tested in this patient as a over some 15 years, undoubtedly decreased the child in view of her frequent and prolonged syncopal perioperative anxiety. Lorazepam was substituted attacks at that time, and was probably not justifiable for diazepam on the second occasion, as it is less during pregnancy. deleterious to the fetus (Whitelaw, Cummings and The pathogenesis of the condition is unclear, but McFadyen, 1981). Callaghan, Nichols and Sweet (1977) advised that is probably the result of an imbalance of tone in the cardiac sympathetic nerves, depressed on the right factors which prolong the Q-T interval should be side or exaggerated on the left. This asymmetry in avoided—hypocalcaemia, hypomagnesaemia and sympathetic tone delays repolarization of the ven- hypokalaemia, as well as certain drugs which protricular myocardium, leading to an increased sus- long the Q-T interval such as quinidine, lignocaine, ceptibility to fibrillation. Recent work suggests that procainamide, and some phenothiazines. They used depression of the right side is more probable. a nitrous oxide, morphine technique with Patients who have had percutaneous left stellate neuromuscular blockade, and stellate ganglion ganglion block (Moss and McDonald, 1971), or block. Incremental analgesic techniques are insurgical left cervico-thoracic gangliectomy (Cal- appropriate in obstetric anaesthesia, and low-dose 1nghnn3 Nichols and Sweet, 1977) showed clinical halothane was used to avoid awareness. The choice improvement with decreases in the frequency of of neuromuscular blocking drug was determined by syncopal attacks and sometimes a decrease in the a wish to avoid tachycardia which, when associated with exercise or emotional stress, can cause venduration of the Q - T interval. dose of propranolol 20 mg and lorazepam 2 mg by mouth 1.5h before operation. Her Englishspeaking sister accompanied her into the anaesthetic room to act as interpreter. Induction and maintenance followed the technique described already. During the somewhat longer procedure (some 70min) she required two increments of suxamethonium 25 mg. After the second there was a decrease in heart rate from 60 beat min"1 to 49 beat min"1 which responded to atropine 0.2 mg i.v.—returning to 78beatmin-1. Two subsequent increments of suxamethonium 10 mg were given with no further alterations in heart rate. Papaveretum lOmg i.v. was given after delivery. Her postoperative course was uneventful and she remains well, continuing to receive propranolol 20 mg twice per day. The second male infant is healthy and has no ECG abnormalities.
JERVELL, LANGE-NIELSON SYNDROME
Ratshin, R. A., Hunt, D., and Russell, R. O. (1971). Q - T interval prolongation, paraoxysmal ventricular arrhythmia* and convulsive syncope. Ann. Intern. Med., 75,919. Whitelaw, A. G., Cummings, A. J.,andMcFadyen,I. R.(1981). The effect of lorazepamon the neoaue. Br. Med. J., 282,1106. Wig, J., Bah', I. M., Single, R. G., Kataria, R. N., and Khattri, H. N. (1979). Prolonged Q - T interval syndrome. Sudden cardiac arrest during anaesthesia. Anaesthesia, 34,37.
ANESTHESIE POUR CESARIENNE ET SYNDROME DE JERVELL, LANGE-NIELSON (SYNDROME DU Q - T LONG)
ACKNOWLEDGEMENTS
The author wishes to thank Dr Matthews, Consultant Cardiologist, at the Western General Hospital, Edinburgh for permission to report on his patient, Dr I. T. Davk, Consultant Anaesthetist at the same hospital for his encouragement and advice, and Mrs I. Blair for typing the manuscript.
RESUME
Cet article decrit le protocole anesthesique utilise chez une patiente porteuse d'un syndrome de Jervell, Lange-Nielson (JLS) et se presentant a deux reprise* pour une cesarienne. Sont egakment discutes ce syndrome, son traitement et le choir de l'anesthesie.
REFERENCES
Brown, M., Iiberthson, R., Ali, H. A., and Lowenstein, E. (1981). Perioperative management of a patient with long Q - T syndrome. Antstktsiology, 55, 586. Callaghan, M. L., Nichols, A. B., and Sweet, R. B. (1977). Anesthetic management of prolonged Q - T interval syndrome. Anestkesiology, 47, 67. Jervell, A., and Lange-Nielson, F. (1957). Congenital deafmutism, functional heart disease with prolongation of the Q - T interval and sudden death. Am. Heart J., 54, 59. Matthews, E. C , Blaint, A. W. jr., and Townsend, J. I. (1972). Q - T prolongation and ventricular arrhythmias with and without drafr"i^ in the same family. Am. J. Cardioi, 29, 702. Medak, R., and Benumof, J. L. (1983). Perioperative management of the prolonged Q - T interval syndrome. Br. J. Anaesth., SS,36\. Mime, J. R., Camm, A. J., and Ward, D. E. (1980). Effect of intravenous propranolol on Q—T interval—a new method of assessment. Br. Heart J., 43, 1. Moss, A. J., and McDonald, J. (1971). Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long Q—T interval syndrome. N. Engl. J. Med., 285, 903. Owitz, S., Pratfla, V., Pratila, M. G., and Dunich, I. (1979). Anaesthetic considerations in the prolonged Q - T interval (LQTS): A case report. Can. Anaesth. Soc. J., 26, 50. Ponte, J., and Lund, J. (1981). Prolongation of the Q - T interval (Romano-Ward syndrome): Anaesthetic management. Br. J. Anaesth.,53,1347.
NARKOSE BEI KAISERSCHNITT UND JERVELL-LANGE-NIELSON-SYNDROM (VERLANGERTES QT-DMTERVALL) ZUSAMMENFASSUNG
Es erfolgt die Beschreibung des anSsthetischen Vorgehens bei zwei Patientinnen mit Jervell-Lange-Nielson-Syndrom wahrend Kaiserschni ft-Opera rionen. Das Syndrom, seine Behandhmg und die Wahl der Anasthesie werden diskutiert.
ANESTESIA PARA OPERAaON CESAREA Y SINDROME DE JERVELL LANGE-NIELSON (SINDROME DEL INTERVALO Q-T PROLONGADO) SUMARIO
Se describe la gestjon anestetica de una paciente con sindrome de Jervell Lange-Nielson (JLN) que se presento dos veces para una operation cesarea. Se discute del sindrome, de su mtamiento y de la sdeccion de la anestesia.
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
tricular fibrillation. Intermittent suxamethonium is a well-tried, safe technique in obstetric anaesthesia. Any bradycardia occurring can be reversed by atropine. This was only required in this patient during the second, longer procedure, and a small dose (0.2 mg) under ECG control restored heart rate to close to its previous value. This patient exhibited no problems in the period after operation, although others have experienced difficulty in managing arrhythmias in uncontrolled patients (Callaghan, Nichols and Sweet, 1977; Owitz et al., 1979; Wigetal., 1979). Our experience supports that of Medak and Benumof (1983) and suggests that the well-controlled patient with prolonged Q-T syndrome may be less at risk than expected. Most reported cases of the prolonged Q-T syndrome fall into the Romano-Ward type. Perhaps the JLN syndrome has a more benign natural history. Improvement with advancing age and a greater susceptibility to control of syncopal attacks by propranolol may render anaesthesia for these patients less hazardous.
657
ANAESTHESIA FOR CAESAREAN SECTION AND THE JERVELL, LANGENIELSON SYNDROME (PROLONGED Q-T INTERVAL SYNDROME) J. V. FRESHWATER SUMMARY
The syndrome of congenital deafness, syncopal attacks and an abnormal ECG showing prolongation of the Q - T interval corrected for heart rate (Q-Tc > 0.45 s) was described first by Jervell and LangeNielson (1957). It is a rare hereditary disease, inheritance being of the autosomal recessive type. The ECG abnormality resembles closely that found in the Romano-Ward syndrome which, however, is not associated with deafness, and has probably an autosomal dominant mode of inheritance. Both may present with a family history of syncope and of sudden death, often in infancy, occurring after exercise or emotional stress. Syncope may occur as a result of Stokes-Adams attacks, asystole or ventricular fibrillation occurring in the presence of atrio-ventricular block. Patients with prolonged Q - T interval syndromes may exhibit the "Torsades des pointes" phenomenon, polymorphic ventricular tachycardia, a rapid ventricular tachycardia characterized by changing QRS morphology. This is often self-terminating, but may degenerate into established ventricular fibrillation. Recent advances in therapy, notably the use of 0-adrenoceptor blocking drugs, have improved survival, and a few patients presenting for surgery and anaesthesia are now recorded in the literature. The patient described is one of the few to have the classic Jervell, Lange-Nielson (JLN) syndrome and, possibly, the first reported to undergo Caesarean section. CASE REPORT
A 20-year-old deaf Sikh female known to have the JLN syndrome presented for the management of her pregnancy. From age Syr she was subject to JANE V. FRESHWATER, M.B., CH.B., F.F.A.R.C.S., Department of
Anaesthetics, Western General Hospital, Crewe Road South, Edinburgh. Present address: Mooklands District General Hospital, Monkscourt Avenue, Airdrie, Lanarkshire.
Stokes-Adams syncopal attacks most frequent during her early childhood. Two siblings had died in infancy. At 9yr the JLN syndrome was diagnosed (Q-Tc 0.53 8) and treatment initiated with digoxin 0.062 5 mg daily. This was later changed to practolol 200 mg twice a day with good control of the attacks. Propranolol 20 mg twice per day was substituted later. On admission to hospital the patient had had no attacks for 3 years in spite of infrequent attendance for follow-up and, probably, poor compliance with therapy. She had an uneventful pregnancy, receiving propranolol 20 mg twice per day and was booked for elective Caesarean section under general anaesthesia. She was admitted for full preoperative assessment, including ECG. Propranolol 20 mg twice per day was continued until the morning of her operation. She was premedicated with diazepam 10 mg by mouth and after pre-oxygenation, cricoid pressure was applied. Anaesthesia was induced with thiopentone 250 mg, and suxamethonium 70 mg given to facilitate tracheal intubation. Ventilation was controlled, and anaesthesia maintain^ with nitrous oxide and 0.5% halothane in oxygen. She received intermittent injections of suxamethonium 50 mg during the 40-min procedure. A live, healthy male infant was delivered which was found to have no ECG abnormality. Immediately following operation the mother received papaveretum 5mg, cyciizine 25mgi.m. and diazepam 2.5 mgi.v. She had shown no additional ECG abnormalities throughout the procedure, and her course after operation was uneventful. Two years later the patient presented for the management of her second pregnancy, and requested sterilization. She had remained wellcontrolled on propranolol 20 mg twice per day, with no further syncopal attacks. She was prepared for general anaesthesia as before, receiving her morning © The Mtrmillan Press Ltd 1984
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
The anaesthetic management is described of a patient with Jervell, Lange-Nielson OLN) syndrome presenting on two occasions for Caesarean section. The syndrome, its treatment and the choice of anaesthesia are discussed.
656
BRITISH JOURNAL OF ANAESTHESIA
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
Propranolol is the treatment of choice. Its beneficial effect is probably not a result of its influence on the Q-T interval, which it tends to prolong, but rather to direct anti-sympathetic activity, and an ability to increase the threshold for ventricular fibrillation (Milne, Camm and Ward, 1980). Unfortunately, not all patients tolerate therapy or are controlled adequately, and further anti-arrhythmic management may be difficult. LJgnocaine and phenytoin have been used following surgery (Brown, et al., 1981). Percutaneous left stellate ganglion block has proved useful (Ponte and Lund, 1981), and surgical sympathectomy could be offered for long-term management. The choice of anaesthetic technique for these patients is difficult. Anaesthesia must be hazardous where there is a risk of a life-threatening arrhythmia. The choice in this patient was further limited by her pregnancy since any technique chosen had to DISCUSSION allay anxiety, provide cardiovascular stability, and JLN syndrome is an autosomal recessive condition. satisfy the strict criteria for safety of mother and Heterozygotes are clinically normal although their child inherent in any obstetric anaesthetic. Communication between the patient and the ECG may show prolongation of the Q-T interval. The frequency in the general population is 1 in medical team was restricted by her severe deafness 300 000 and in children with perceptive deafness, 1 and language problems. This difficulty and the risks in 100. Consanguinity of parents predisposes to the of possible hypotension in the presence of 0syndrome (Matthews, Blaint and Townsend, 1972): blockade excluded extradural anaesthesia as a suitaSikhs usually inter-marry within their own com- ble technique. General anaesthesia, using a well munity. The frequency of the syncopal attacks di- established technique and careful monitoring, minishes with age (Ratshin, Hunt and Russell, offered the safest choice. 1971). Syncope in patients with prolongation of the Preoperative visits, premedicants, the presence of Q - T interval is usually related to exercise; in this a familiar relative in the induction room, and the patient emotional stress was a precipitating factor. excellent rapport between patient and cardiologist Exercise tolerance was not tested in this patient as a over some 15 years, undoubtedly decreased the child in view of her frequent and prolonged syncopal perioperative anxiety. Lorazepam was substituted attacks at that time, and was probably not justifiable for diazepam on the second occasion, as it is less during pregnancy. deleterious to the fetus (Whitelaw, Cummings and The pathogenesis of the condition is unclear, but McFadyen, 1981). Callaghan, Nichols and Sweet (1977) advised that is probably the result of an imbalance of tone in the cardiac sympathetic nerves, depressed on the right factors which prolong the Q-T interval should be side or exaggerated on the left. This asymmetry in avoided—hypocalcaemia, hypomagnesaemia and sympathetic tone delays repolarization of the ven- hypokalaemia, as well as certain drugs which protricular myocardium, leading to an increased sus- long the Q-T interval such as quinidine, lignocaine, ceptibility to fibrillation. Recent work suggests that procainamide, and some phenothiazines. They used depression of the right side is more probable. a nitrous oxide, morphine technique with Patients who have had percutaneous left stellate neuromuscular blockade, and stellate ganglion ganglion block (Moss and McDonald, 1971), or block. Incremental analgesic techniques are insurgical left cervico-thoracic gangliectomy (Cal- appropriate in obstetric anaesthesia, and low-dose 1nghnn3 Nichols and Sweet, 1977) showed clinical halothane was used to avoid awareness. The choice improvement with decreases in the frequency of of neuromuscular blocking drug was determined by syncopal attacks and sometimes a decrease in the a wish to avoid tachycardia which, when associated with exercise or emotional stress, can cause venduration of the Q - T interval. dose of propranolol 20 mg and lorazepam 2 mg by mouth 1.5h before operation. Her Englishspeaking sister accompanied her into the anaesthetic room to act as interpreter. Induction and maintenance followed the technique described already. During the somewhat longer procedure (some 70min) she required two increments of suxamethonium 25 mg. After the second there was a decrease in heart rate from 60 beat min"1 to 49 beat min"1 which responded to atropine 0.2 mg i.v.—returning to 78beatmin-1. Two subsequent increments of suxamethonium 10 mg were given with no further alterations in heart rate. Papaveretum lOmg i.v. was given after delivery. Her postoperative course was uneventful and she remains well, continuing to receive propranolol 20 mg twice per day. The second male infant is healthy and has no ECG abnormalities.
JERVELL, LANGE-NIELSON SYNDROME
Ratshin, R. A., Hunt, D., and Russell, R. O. (1971). Q - T interval prolongation, paraoxysmal ventricular arrhythmia* and convulsive syncope. Ann. Intern. Med., 75,919. Whitelaw, A. G., Cummings, A. J.,andMcFadyen,I. R.(1981). The effect of lorazepamon the neoaue. Br. Med. J., 282,1106. Wig, J., Bah', I. M., Single, R. G., Kataria, R. N., and Khattri, H. N. (1979). Prolonged Q - T interval syndrome. Sudden cardiac arrest during anaesthesia. Anaesthesia, 34,37.
ANESTHESIE POUR CESARIENNE ET SYNDROME DE JERVELL, LANGE-NIELSON (SYNDROME DU Q - T LONG)
ACKNOWLEDGEMENTS
The author wishes to thank Dr Matthews, Consultant Cardiologist, at the Western General Hospital, Edinburgh for permission to report on his patient, Dr I. T. Davk, Consultant Anaesthetist at the same hospital for his encouragement and advice, and Mrs I. Blair for typing the manuscript.
RESUME
Cet article decrit le protocole anesthesique utilise chez une patiente porteuse d'un syndrome de Jervell, Lange-Nielson (JLS) et se presentant a deux reprise* pour une cesarienne. Sont egakment discutes ce syndrome, son traitement et le choir de l'anesthesie.
REFERENCES
Brown, M., Iiberthson, R., Ali, H. A., and Lowenstein, E. (1981). Perioperative management of a patient with long Q - T syndrome. Antstktsiology, 55, 586. Callaghan, M. L., Nichols, A. B., and Sweet, R. B. (1977). Anesthetic management of prolonged Q - T interval syndrome. Anestkesiology, 47, 67. Jervell, A., and Lange-Nielson, F. (1957). Congenital deafmutism, functional heart disease with prolongation of the Q - T interval and sudden death. Am. Heart J., 54, 59. Matthews, E. C , Blaint, A. W. jr., and Townsend, J. I. (1972). Q - T prolongation and ventricular arrhythmias with and without drafr"i^ in the same family. Am. J. Cardioi, 29, 702. Medak, R., and Benumof, J. L. (1983). Perioperative management of the prolonged Q - T interval syndrome. Br. J. Anaesth., SS,36\. Mime, J. R., Camm, A. J., and Ward, D. E. (1980). Effect of intravenous propranolol on Q—T interval—a new method of assessment. Br. Heart J., 43, 1. Moss, A. J., and McDonald, J. (1971). Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long Q—T interval syndrome. N. Engl. J. Med., 285, 903. Owitz, S., Pratfla, V., Pratila, M. G., and Dunich, I. (1979). Anaesthetic considerations in the prolonged Q - T interval (LQTS): A case report. Can. Anaesth. Soc. J., 26, 50. Ponte, J., and Lund, J. (1981). Prolongation of the Q - T interval (Romano-Ward syndrome): Anaesthetic management. Br. J. Anaesth.,53,1347.
NARKOSE BEI KAISERSCHNITT UND JERVELL-LANGE-NIELSON-SYNDROM (VERLANGERTES QT-DMTERVALL) ZUSAMMENFASSUNG
Es erfolgt die Beschreibung des anSsthetischen Vorgehens bei zwei Patientinnen mit Jervell-Lange-Nielson-Syndrom wahrend Kaiserschni ft-Opera rionen. Das Syndrom, seine Behandhmg und die Wahl der Anasthesie werden diskutiert.
ANESTESIA PARA OPERAaON CESAREA Y SINDROME DE JERVELL LANGE-NIELSON (SINDROME DEL INTERVALO Q-T PROLONGADO) SUMARIO
Se describe la gestjon anestetica de una paciente con sindrome de Jervell Lange-Nielson (JLN) que se presento dos veces para una operation cesarea. Se discute del sindrome, de su mtamiento y de la sdeccion de la anestesia.
Downloaded from http://bja.oxfordjournals.org/ at University of California, Santa Barbara on July 2, 2015
tricular fibrillation. Intermittent suxamethonium is a well-tried, safe technique in obstetric anaesthesia. Any bradycardia occurring can be reversed by atropine. This was only required in this patient during the second, longer procedure, and a small dose (0.2 mg) under ECG control restored heart rate to close to its previous value. This patient exhibited no problems in the period after operation, although others have experienced difficulty in managing arrhythmias in uncontrolled patients (Callaghan, Nichols and Sweet, 1977; Owitz et al., 1979; Wigetal., 1979). Our experience supports that of Medak and Benumof (1983) and suggests that the well-controlled patient with prolonged Q-T syndrome may be less at risk than expected. Most reported cases of the prolonged Q-T syndrome fall into the Romano-Ward type. Perhaps the JLN syndrome has a more benign natural history. Improvement with advancing age and a greater susceptibility to control of syncopal attacks by propranolol may render anaesthesia for these patients less hazardous.
657