Anaesthesia, 1979, Volume 34, pages 37-40 CASE REPORT
Prolonged Q-T interval syndrome Sudden cardiac arrest during anaesthesia
J. W I G , I.M. BALI, R.G. SINGH, R.N. KATARIA H.N. KHATTRI
I n 1957, Jerwell & Lange-Nielson' described a syndrome of congenital deafness, prolonged Q-T interval, syncopal attacks due to ventricular fibrillation and sudden death. Six years later Romano2 and Ward3 published a description of a similar syndrome without a hearing defect but with an autosomal dominant rather than recessive inheritance. Probably because of lack of awareness of this entity, only 220 cases are described in the literature to date.4 The oldest survival reported was 45 years of age.5 Electrocardiogram (ECG) and clinical preseriration. The Q-T interval represents one complete ventricular contraction including the electrical phase of depolarisation and repolarisation. It extends from the beginning of the QRS complex to the end of the 'T' wave (Fig. 1) and can be measured in either a limb or precordial lead. The normal value at physiological heart rates is less than 0 . 4 3 ~ ~Mootharts ~' described three modes ofpresentation of this entity; first transient episodes of palpitation, numbness and anginal type of chest pain without loss of consciousness; secondly, sudden loss of consciousness usually associated with exertion or emotional stress; thirdly, sudden death. A useful diagnostic test is the 'Treadmill Test' which results in an increase in Q-T interval which is short lived. Abnormal T wave changes with superimposition of P wave on T are also present.8 Pathogenesis. Prolonged Q-T interval syn-
AND
drome can be classified as ~ongenitall-~ and the acquired ~ a r i e t y In . ~ the acquired type the syncopal attacks, due to ventricular fibrillation, occur independently of the emotional state or physical exertion and are always secondary to myocarditis, myocardial infarct, atrio-ventricular block, sinus bradycardia, hypokalaemia, hypocalcaemia and overdosage of drugs like quinidine, procainamide, imipramine, amidoran and phenothiazines. The ECG in the childhood of these patients is normal. Hypothesis for the aetiology of' congenital Q-T syndrome. Various hypotheses have been put forward to explain this syndrome. Asymmetrical adrenergic stimulation of the heart ''*I is the most accepted of the hypotheses and similar changes can be reproduced by asymmetrical alteration in the sympathetic tone. The best results are obtained by giving B-blockers or by blocking the left sympathetic stellate ganglion along with the first thoracic g a n g l i ~ n . ' ~ . ' The level of this imbalance is unknown.14 Abnormality in the vascularisation of the sinus node has also been implicated,' whilst abnormality of the Purkinje fibres has been blamed by Jerwell,'6 and ~ t h e r s ~ - consider ~*" the defect to be an abnormality of myocardial metabolism. Shen & Jennings's advocate myocardial influx of calcium as the cause and others believe that potassium and calcium deficiency are important causes.
J. Wig, MD, Lecturer in Anaesthetics, I.M. Bali, MS(Surg), MS (Anaesth), PhD, FFARCS, Associate Professor in Anaesthetics, R.G.Singh, MD, Senior Resident in Medicine, R.N.Kataria, FRCS, Associate Professor in Surgery and H.N. Khattri, MRCP, Assistant Professor in Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh, India. 0003-2409/79/0100-0037S02.00
0 1979 Blackwell
Scientific Publications
37
38
J. Wig el al. I
I
I I
F
Y
Normal 0-TC0.4 s
I
.
t
.
P Prolonged Q - T 0.7 s
Fig. 1. Normal and prolonged Q-T tracings.
Case report
A 50 year old woman, with a diagnosis of hydatid cyst of the liver of 30 years duration, presented for laparotomy. There was no history of previous illness and no past history of syncopal attacks or deafness and she was not receiving any drugs. There was no family history of similar complaints. On examination the patient was thin, BP 110/70 mmHg. pulse rate 60/min, regular and good volume. Cardiovascular and respiratory systems were essentially normal. The haemoglobin level was 8.2 g%, serum electrolytes and serum proteins were within normal limits. The serum bilirubin was 2 mg%. A Casoni test for hydatid disease was positive. A skiagram of the chest was normal and an ECG showed a prolonged Q-T interval of 0.7 s (Fig. 2).
Anaesthesia
The patient was premedicated with diazepam 5 mg orally the night before and 5 me, 90 min prior to surgery. Induction of anaesthesia was performed with electrocardiographic control, and after pre-oxygenating the patient, thiopentone sodium (2.5 %) 125 mg was administered and tracheal intubation performed after the intravenous injection of gallamine 120 mg. Anaesthesia was maintained with 50% nitrous oxide in oxygen and 0.5% halothane using a 'Fluotec' mark 111 vapourizer. Analgesia was supplemented with intravenous morphine 4 mg. An additional 40 mg gallamine was given as a supplement. Surgery was started 20 min after induction of anaesthesia and cardiac arrest noted 26 min. later, 46 min after induction of anaesthesia.
Prolonged Q-T interual syndrome
39
Fig. 2. The patients' ECG showing prolonged Q-T interval.
During the period from induction to arrest, no ECG changes were observed except sinus tachycardia of lOO/min after intubation, which settled after 5 min. As the hydatid cyst was being aspirated sudden cardiac arrest was observed. The ECG trace showed arrest in asystole. Adrenaline 2 ml of 1:10,000 was given intravenously and repeated 5 min later. The following drugs were given during the resuscitation; hydrocortisone 1 g intravenously, sodium bicarbonate 100 mEquiv., and calcium gluconate 2000 mg, as a lo?! solution. Simultaneously internal cardiac massage through the diaphragm was started. Twice the heart returned to ventricular fibrillation, and DC defibrillation resulted in asystole. Resuscitative measures were continued for one hour after the arrest. At no time during resuscitation was there an effective myocardial contraction.
Discussion Prolonged Q-T interval syndrome, as is evident from the literature, is a very serious and suddenly fatal condition and presents major problems of management to the anaesthetist. A simple yet effective preanaesthetic measure in such cases is to administer 8 blockers.10*11,14 B blockers are reputed to have reduced the mortality rate from 64 to 6%.14 While administering these drugs, if the heart rate falls below 60 per min, interval transvenous pacing may be required to control the bradycardia. If medical treatment fails, left stellate ganglion block has been advised1**' in an effort to minimise or prevent the
asymmetric adrenergic stimulation of the heart. This procedure has been claimed to have had complete success in three cases of prolonged Q-T interval syndrome.I4 In the acquired variety of Q-T prolongation, the primary pathology must be. treated effectively before any anaesthetic procedure is undertaken. The case presented probably belongs to the congenital variety of prolonged Q-T interval syndrome as no other obvious cause for classifying it as the acquired type could be found. The absence of syncopal attacks and loss of hearing makes it a rare case. Lack of awareness of the syndrome and therefore inadequate preanaesthetic preparation of this case was the cause of the sudden cardiac arrest. The lack of response to the resuscitative measures carried out in this patient alsopoints to the difficulty in reversing the cardiac arrest should this occur.5 The correct anaesthetic management of such a case should include the production of a calm, anxiety free state in the patient and a left stellate ganglion block or 8-blocker administration with internal pacing performed prior to anaesthesia. Effective treatment of conditions aggravating a prolonged Q-T interval such as hypokalaemia, hypocalcaemia and hypomagnesaemia should be undertaken and drugs like procainamide, lignocaine and the phenothiazines should be avoided. Induction of anaesthesia should be without incident and deeper planes maintained without causing hypoxia, hypercarbia or hypotension. Adequate postoperative analgesia must be arranged and a defibrillator should be at hand at all times.
40
J. Wig et al. Summary
A review of the literature concerning the causation of prolonged Q-T interval syndrome is presented. A fatal case, 50 years of age, with
this rare entity is also reported, in whom a sudden cardiac arrest occurred 46 min after induction of anaesthesia. An outline for the successful anaesthetic management of patients with prolonged Q-T interval syndrome is suggested.
Key words COMPLICATIONS; arrest, cardiac. HEART; conduction, congenital defect.
Rcferences I . JERWELL,A. & LANOE-NIELSON, F. (1957)Congenital dcaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. American Heart Joiirnal, 54, 59. 2. ROMANO. C., GENME, G. & PONOIGLIONE, R. (1963) Aritmie cardiache rare dell’eta pediatrica. 11. Clinica Pediatrica (Bologna), 45, 656. 3. WARD,O.C. (1964) A new familial cardiac syndrome in children. Joiirnal ofthe Irish Medical Association, 54, 103. P.J., PERITI,M. & MALLIANI, A. (1975) 4. SCHWARTZ, The long Q-T syndrome. American HeartJournal, 89, 378. 5 . SCHNEIDER, R.R., BAHLER,A,, PINCUS,J. & STIMMEL,B. (1977) Asymptomatic idiopathic syndrome of prolonged Q-T interval in a 45-yearold woman. Chest, 71, 210. 6. ASHMAN. R. (1939) Normal duration of the Q-T interval. Proceedings of the Society for Experimental Biology and Medicine, 40,150.
7. SIMONSON, M.E., CADY,L.D. JR, & WOODBURY, M. (1962) The normal Q-T interval. American Heart Journal, 63, 747. 8. MOOTHART,R.W., PRYOR,R., HAWLEY,R.L., CLIFFORD, N.J. & BLOUNT,G.S. JR. (1976) The heritable syndrome of prolonged Q-T interval, syncope and sudden death. Electron microscopic observations. Chest, 70, 263. P., ABITOL,G., DESSEKTENNE, 9. Morrd, G., COUMEL, F. & SLAMA,R. (1970) Le syndrome Q-T longet syncopes par “torsades de pointe”. Archives des Maladies du Coeur, 63, 831. 10. OLLEY,P.M. & FOWLER, R.S.(1970) The surdocardiac syndrome and therapeutic observations. British Heart Journal, 32, 467. 11. DI SALLE, E., BAKER, K.M., BAREGGI,S.R., WATKINS,W.D., CHIDSEY,C.A., FRIGERIO, A. & MORSELLI, P.L. (1973) A sensitive gas chromatographio method for the determination of propranolol in human plasma. Journal of Chromatography, 84, 347. J. (1971) Unilateral 12. Moss, A.J. & MCDONALD, cervicothoracic sympathetic ganglionectomy for the treatment of long Q-T interval syndrome. New England Journal of Medicine, 285, 903. 13. DEAR,W. (1974) Personal communication. 14. SCHWARTZ, P.J. (1973) The role of B-blockers in the therapy of the long Q-T syndrome. Symposium 00 B-blocking Agents, Alghero, Italy, 1973 (Ed. by E. Tajoli). 15. JAMES, T.N. (1969) Q-T prolongation and sudden death. Modern Concepts of Cardiooascular Disease, 38, 35. 16. JERWELL, A., THINGSTAD, R. & ENDSJO,T. (1966) The surdocardiac syndrome: three new cases of congenital deafness with syncopal attacks and Q-T prolongation in the electrocardiogram. American Heart Journal, 73, 582. 17. JERWELL,A. (1971) Surdocardiac and related syndromes in children. Adiiances in Internal Medicine, 17,425. 18. SHEN,A.C. & JENNINGS, R.B. (1972) Myocardial calcium and magnesium in acute ischemic injury. American Journal of Pathology, 67,417.
Prolonged Q-T interval syndrome Sudden cardiac arrest during anaesthesia
J. W I G , I.M. BALI, R.G. SINGH, R.N. KATARIA H.N. KHATTRI
I n 1957, Jerwell & Lange-Nielson' described a syndrome of congenital deafness, prolonged Q-T interval, syncopal attacks due to ventricular fibrillation and sudden death. Six years later Romano2 and Ward3 published a description of a similar syndrome without a hearing defect but with an autosomal dominant rather than recessive inheritance. Probably because of lack of awareness of this entity, only 220 cases are described in the literature to date.4 The oldest survival reported was 45 years of age.5 Electrocardiogram (ECG) and clinical preseriration. The Q-T interval represents one complete ventricular contraction including the electrical phase of depolarisation and repolarisation. It extends from the beginning of the QRS complex to the end of the 'T' wave (Fig. 1) and can be measured in either a limb or precordial lead. The normal value at physiological heart rates is less than 0 . 4 3 ~ ~Mootharts ~' described three modes ofpresentation of this entity; first transient episodes of palpitation, numbness and anginal type of chest pain without loss of consciousness; secondly, sudden loss of consciousness usually associated with exertion or emotional stress; thirdly, sudden death. A useful diagnostic test is the 'Treadmill Test' which results in an increase in Q-T interval which is short lived. Abnormal T wave changes with superimposition of P wave on T are also present.8 Pathogenesis. Prolonged Q-T interval syn-
AND
drome can be classified as ~ongenitall-~ and the acquired ~ a r i e t y In . ~ the acquired type the syncopal attacks, due to ventricular fibrillation, occur independently of the emotional state or physical exertion and are always secondary to myocarditis, myocardial infarct, atrio-ventricular block, sinus bradycardia, hypokalaemia, hypocalcaemia and overdosage of drugs like quinidine, procainamide, imipramine, amidoran and phenothiazines. The ECG in the childhood of these patients is normal. Hypothesis for the aetiology of' congenital Q-T syndrome. Various hypotheses have been put forward to explain this syndrome. Asymmetrical adrenergic stimulation of the heart ''*I is the most accepted of the hypotheses and similar changes can be reproduced by asymmetrical alteration in the sympathetic tone. The best results are obtained by giving B-blockers or by blocking the left sympathetic stellate ganglion along with the first thoracic g a n g l i ~ n . ' ~ . ' The level of this imbalance is unknown.14 Abnormality in the vascularisation of the sinus node has also been implicated,' whilst abnormality of the Purkinje fibres has been blamed by Jerwell,'6 and ~ t h e r s ~ - consider ~*" the defect to be an abnormality of myocardial metabolism. Shen & Jennings's advocate myocardial influx of calcium as the cause and others believe that potassium and calcium deficiency are important causes.
J. Wig, MD, Lecturer in Anaesthetics, I.M. Bali, MS(Surg), MS (Anaesth), PhD, FFARCS, Associate Professor in Anaesthetics, R.G.Singh, MD, Senior Resident in Medicine, R.N.Kataria, FRCS, Associate Professor in Surgery and H.N. Khattri, MRCP, Assistant Professor in Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh, India. 0003-2409/79/0100-0037S02.00
0 1979 Blackwell
Scientific Publications
37
38
J. Wig el al. I
I
I I
F
Y
Normal 0-TC0.4 s
I
.
t
.
P Prolonged Q - T 0.7 s
Fig. 1. Normal and prolonged Q-T tracings.
Case report
A 50 year old woman, with a diagnosis of hydatid cyst of the liver of 30 years duration, presented for laparotomy. There was no history of previous illness and no past history of syncopal attacks or deafness and she was not receiving any drugs. There was no family history of similar complaints. On examination the patient was thin, BP 110/70 mmHg. pulse rate 60/min, regular and good volume. Cardiovascular and respiratory systems were essentially normal. The haemoglobin level was 8.2 g%, serum electrolytes and serum proteins were within normal limits. The serum bilirubin was 2 mg%. A Casoni test for hydatid disease was positive. A skiagram of the chest was normal and an ECG showed a prolonged Q-T interval of 0.7 s (Fig. 2).
Anaesthesia
The patient was premedicated with diazepam 5 mg orally the night before and 5 me, 90 min prior to surgery. Induction of anaesthesia was performed with electrocardiographic control, and after pre-oxygenating the patient, thiopentone sodium (2.5 %) 125 mg was administered and tracheal intubation performed after the intravenous injection of gallamine 120 mg. Anaesthesia was maintained with 50% nitrous oxide in oxygen and 0.5% halothane using a 'Fluotec' mark 111 vapourizer. Analgesia was supplemented with intravenous morphine 4 mg. An additional 40 mg gallamine was given as a supplement. Surgery was started 20 min after induction of anaesthesia and cardiac arrest noted 26 min. later, 46 min after induction of anaesthesia.
Prolonged Q-T interual syndrome
39
Fig. 2. The patients' ECG showing prolonged Q-T interval.
During the period from induction to arrest, no ECG changes were observed except sinus tachycardia of lOO/min after intubation, which settled after 5 min. As the hydatid cyst was being aspirated sudden cardiac arrest was observed. The ECG trace showed arrest in asystole. Adrenaline 2 ml of 1:10,000 was given intravenously and repeated 5 min later. The following drugs were given during the resuscitation; hydrocortisone 1 g intravenously, sodium bicarbonate 100 mEquiv., and calcium gluconate 2000 mg, as a lo?! solution. Simultaneously internal cardiac massage through the diaphragm was started. Twice the heart returned to ventricular fibrillation, and DC defibrillation resulted in asystole. Resuscitative measures were continued for one hour after the arrest. At no time during resuscitation was there an effective myocardial contraction.
Discussion Prolonged Q-T interval syndrome, as is evident from the literature, is a very serious and suddenly fatal condition and presents major problems of management to the anaesthetist. A simple yet effective preanaesthetic measure in such cases is to administer 8 blockers.10*11,14 B blockers are reputed to have reduced the mortality rate from 64 to 6%.14 While administering these drugs, if the heart rate falls below 60 per min, interval transvenous pacing may be required to control the bradycardia. If medical treatment fails, left stellate ganglion block has been advised1**' in an effort to minimise or prevent the
asymmetric adrenergic stimulation of the heart. This procedure has been claimed to have had complete success in three cases of prolonged Q-T interval syndrome.I4 In the acquired variety of Q-T prolongation, the primary pathology must be. treated effectively before any anaesthetic procedure is undertaken. The case presented probably belongs to the congenital variety of prolonged Q-T interval syndrome as no other obvious cause for classifying it as the acquired type could be found. The absence of syncopal attacks and loss of hearing makes it a rare case. Lack of awareness of the syndrome and therefore inadequate preanaesthetic preparation of this case was the cause of the sudden cardiac arrest. The lack of response to the resuscitative measures carried out in this patient alsopoints to the difficulty in reversing the cardiac arrest should this occur.5 The correct anaesthetic management of such a case should include the production of a calm, anxiety free state in the patient and a left stellate ganglion block or 8-blocker administration with internal pacing performed prior to anaesthesia. Effective treatment of conditions aggravating a prolonged Q-T interval such as hypokalaemia, hypocalcaemia and hypomagnesaemia should be undertaken and drugs like procainamide, lignocaine and the phenothiazines should be avoided. Induction of anaesthesia should be without incident and deeper planes maintained without causing hypoxia, hypercarbia or hypotension. Adequate postoperative analgesia must be arranged and a defibrillator should be at hand at all times.
40
J. Wig et al. Summary
A review of the literature concerning the causation of prolonged Q-T interval syndrome is presented. A fatal case, 50 years of age, with
this rare entity is also reported, in whom a sudden cardiac arrest occurred 46 min after induction of anaesthesia. An outline for the successful anaesthetic management of patients with prolonged Q-T interval syndrome is suggested.
Key words COMPLICATIONS; arrest, cardiac. HEART; conduction, congenital defect.
Rcferences I . JERWELL,A. & LANOE-NIELSON, F. (1957)Congenital dcaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. American Heart Joiirnal, 54, 59. 2. ROMANO. C., GENME, G. & PONOIGLIONE, R. (1963) Aritmie cardiache rare dell’eta pediatrica. 11. Clinica Pediatrica (Bologna), 45, 656. 3. WARD,O.C. (1964) A new familial cardiac syndrome in children. Joiirnal ofthe Irish Medical Association, 54, 103. P.J., PERITI,M. & MALLIANI, A. (1975) 4. SCHWARTZ, The long Q-T syndrome. American HeartJournal, 89, 378. 5 . SCHNEIDER, R.R., BAHLER,A,, PINCUS,J. & STIMMEL,B. (1977) Asymptomatic idiopathic syndrome of prolonged Q-T interval in a 45-yearold woman. Chest, 71, 210. 6. ASHMAN. R. (1939) Normal duration of the Q-T interval. Proceedings of the Society for Experimental Biology and Medicine, 40,150.
7. SIMONSON, M.E., CADY,L.D. JR, & WOODBURY, M. (1962) The normal Q-T interval. American Heart Journal, 63, 747. 8. MOOTHART,R.W., PRYOR,R., HAWLEY,R.L., CLIFFORD, N.J. & BLOUNT,G.S. JR. (1976) The heritable syndrome of prolonged Q-T interval, syncope and sudden death. Electron microscopic observations. Chest, 70, 263. P., ABITOL,G., DESSEKTENNE, 9. Morrd, G., COUMEL, F. & SLAMA,R. (1970) Le syndrome Q-T longet syncopes par “torsades de pointe”. Archives des Maladies du Coeur, 63, 831. 10. OLLEY,P.M. & FOWLER, R.S.(1970) The surdocardiac syndrome and therapeutic observations. British Heart Journal, 32, 467. 11. DI SALLE, E., BAKER, K.M., BAREGGI,S.R., WATKINS,W.D., CHIDSEY,C.A., FRIGERIO, A. & MORSELLI, P.L. (1973) A sensitive gas chromatographio method for the determination of propranolol in human plasma. Journal of Chromatography, 84, 347. J. (1971) Unilateral 12. Moss, A.J. & MCDONALD, cervicothoracic sympathetic ganglionectomy for the treatment of long Q-T interval syndrome. New England Journal of Medicine, 285, 903. 13. DEAR,W. (1974) Personal communication. 14. SCHWARTZ, P.J. (1973) The role of B-blockers in the therapy of the long Q-T syndrome. Symposium 00 B-blocking Agents, Alghero, Italy, 1973 (Ed. by E. Tajoli). 15. JAMES, T.N. (1969) Q-T prolongation and sudden death. Modern Concepts of Cardiooascular Disease, 38, 35. 16. JERWELL, A., THINGSTAD, R. & ENDSJO,T. (1966) The surdocardiac syndrome: three new cases of congenital deafness with syncopal attacks and Q-T prolongation in the electrocardiogram. American Heart Journal, 73, 582. 17. JERWELL,A. (1971) Surdocardiac and related syndromes in children. Adiiances in Internal Medicine, 17,425. 18. SHEN,A.C. & JENNINGS, R.B. (1972) Myocardial calcium and magnesium in acute ischemic injury. American Journal of Pathology, 67,417.
