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Year : 2021  |  Volume : 15  |  Issue : 1  |  Page : 149-151  

Anesthesia for a child with congenital long QT syndrome, a case report and literature review

1 Department of Anesthesiology and Reanimation, Faculty of Medicine, University of Eskişehir Osmangazi, Eskişehir, Turkey
2 Department of Otolaryngology, Faculty of Medicine, University of Eskişehir Osmangazi, Eskişehir, Turkey

Date of Submission24-Mar-2021
Date of Acceptance06-Jun-2021
Date of Web Publication30-Aug-2021

Correspondence Address:
Assoc. Prof. Dr. Ferda Yaman
Department of Anesthesiology and Reanimation, Faculty of Medicine, University of Eskişehir Osmangazi, Eskişehir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aer.aer_48_21

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Long QT syndrome is an inherited disorder of the heart's electrical activity that may also be associated with malignant arrhythmia and cause sudden death. In addition to this inherited condition, several commonly used anesthetic drugs can prolong the QT interval. We present here a 17-month-old male patient who underwent general anesthesia for a cochlear implant. No cardiac arrhythmia was observed in the patient, whose muscle relaxant effect was reversed using sugammadex. The application of intravenous anesthetics was preferred to maintain anesthesia for this patient and was safely applied.

Keywords: Cochlear implant, congenital long QT syndrome, pediatric anesthesia, rocuronium, sugammadex

How to cite this article:
Yaman F, Baydogan N, Bilir A, Incesulu A. Anesthesia for a child with congenital long QT syndrome, a case report and literature review. Anesth Essays Res 2021;15:149-51

How to cite this URL:
Yaman F, Baydogan N, Bilir A, Incesulu A. Anesthesia for a child with congenital long QT syndrome, a case report and literature review. Anesth Essays Res [serial online] 2021 [cited 2021 Nov 30];15:149-51. Available from:

   Introduction Top

Long QT syndrome (LQTS) is an inherited condition that causes primary arrhythmia and that affects 1 out of every 2000 otherwise healthy babies.[1] The condition is characterized by the slow depolarization of the cardiac ion channel, which is observed on an electrocardiogram (ECG) as a prolonged QT interval, secondary to a delayed repolarization of the action potential on the cardiomyocyte membrane.[2] Sudden cardiac death is observed in 10%–12% of patients with LQTS, and a mortality rate of 21% is expected within 1 year of the first syncopal episode; however, there is treatment that can dramatically reduce these risks.[3],[4] A recessive form of LQTS was identified by Jervell and Lange-Nielsen in 1957 to be associated with a child's deafness, and an autosomal dominant familial form was identified by Romano in 1963 and Conor Ward in 1964. Schwartz and Locati (1985) were the first to publish studies on the natural history of the disease and noted a 71% mortality rate in patients who were left untreated after the first syncope. That mortality rate has since been greatly reduced to 0.3% with appropriate medical treatment.[4],[5]

The QT interval can also be prolonged by several commonly used anesthetic drugs. During the perioperative period, frequent and sudden increases in sympathetic stimulation combined with administration of drugs that prolong the QT interval can create a substrate for the development of malignant arrhythmias. We report here a 17-month-old male patient who underwent general anesthesia for a cochlear implant with LQTS. The child's family has given written informed consent to the publication of this case report. This article conforms to relevant CAse REport (CARE) guidelines.

   Case Report Top

With the family's permission to study and report in the literature, a 17-month-old male patient with bilateral sensorineural hearing loss with congenital LQTS was to undergo a cochlear implant and referred to the anesthesia outpatient clinic. From information gathered from the patient's relatives, we learned that the father, brother, grandfather aunt, and close relatives also had LQTS and that the younger brother was physically and hearing impaired and had already had bilateral cochlear and pacemaker implants. An older brother had no medical abnormalities. The patient had a history of syncope at 37 day, and the causes were investigated by pediatric cardiologists because of the family history. The patient's echocardiograph showed a normal ejection fraction, and he was prescribed 3−1.d−1 propranolol HCl (Dideral®) after a diagnosis of LQTS based on the prolonged QT interval observed on his ECG (QT = 0.459), and it was noted that the patient complied with his medication regularly. There was no cardiological inconvenience for the implant surgery as long as there was close monitoring, a supply of intravenous (iv) magnesium on hand, anesthetics that did not prolong the QT interval, and continued us of beta-blockers. In the preoperative patient evaluation, the vital signs were normal, mouth opening was good, there was no restriction in head-and-neck movements, and there were no pathologies in the other physical examination findings. Laboratory tests were within normal ranges. Relatives of the patient were informed about the risks of anesthesia, and informed patient consent was obtained from them for the intervention. The patient was not premedicated on the morning of the intervention. Sevoflurane induction was applied to the patient for vascular access. A defibrillator, which was checked for use on pediatric patients, was made available in the operating room. After opening the vascular access route, 1−1 lidocaine, 3−1 propofol, 0.5 μ−1 remifentanil, and 0.5−1 rocuronium bromide were administered intravenously in induction. During maintenance, 50 μ−1.min−1 propofol along with a mixture of 50% oxygen and 50% air and 0.05−1.min−1 remifentanil infusion (total iv anesthesia [TIVA]) were applied. The surgical team was warned about the use of local anesthesia without adrenaline. No complications were observed during the induction and surgical procedure. The patient's vital signs remained stable, and there was no rhythm or interval changes during heart monitoring. No hypotension was encountered. The vasoactive drugs are not safe. Phenylephrine and metaraminol may be chosen but phenylephrine and metaraminol are not available in Turkey. Hence, norepinephrine was prepared if needed the dose should be titrated.

The intervention time was recorded as 2 h 40 min. Toward the end of the surgical procedure, for postoperative pain, 120 mg paracetamol and 15 mg phenobarbital (Paranox®) rectally administered. TIVA was discontinued and the effect of the muscle relaxant was reversed by giving 2−1 sugammadex intravenously. The patient was taken to the recovery room after comfortable awakening and extubation and was monitored for 2 h at the yard of otorhinolaryngology to reduce the patient's anxiety about separation from the family, and monitoring was done carefully. The patient did not experience any medical problems after the surgery. The surgical service doctor reported no problems in service follow-up.

   Discussion Top

Jervell and Lange-Nielsen syndrome is an autosomal recessive rare form of LQTS that affects between 1 in 1,000,000 and 4,000,000 people, is highly malignant, and is associated with bilateral sensorineural deafness. This syndrome was first characterized in 1957 by Jervell and Lange-Nielsen, who described a family comprising 4 of 10 children who suffered from deafness and sudden syncopal spells. Three of the four children demonstrated a prolonged QT interval on their ECGs; the fourth child died before an ECG could be obtained.[6] The congenital deafness associated with Jervell and Lange-Nielsen syndrome occurs only when two mutant genes are inherited; these mutant genes are known to impair electrical conduction of the auditory nerves and are associated with a more severe form of the disease and increased risk of sudden cardiac death. Genetic testing was not conducted on the patients we studied; diagnosis of LQTS was made when the patient was 37 days old after experiencing syncope and based on a family history of the condition.

During the preoperative evaluation, any electrolytes given should be evaluated because abnormalities, such as hypokalemia, hypomagnesemia, and hypocalcemia, associated with them can increase the risk of delayed repolarization and may predispose patients to develop ventricular arrhythmias. Hypokalemia and hypocalcemia are independent risk factors associated with a prolonged QT interval. Although electrolytes should be monitored closely, it is reasonable to consider treatment with 30−1 magnesium given its low risk of toxicity and stabilizing effect on the myocardium.[7] There was no electrolyte imbalance in this case, but magnesium was prepared for possible ventricular tachyarrhythmia (torsades de pointes [TdP]).

In a study of 158 anesthetics used on patients with LQTS, volatile agents were used to some degree in 81% of cases and their use was not related to the development of TdP. In practice, many published accounts of anesthesia used on children with LQTS, including the largest published case series comprising 103 children, demonstrated that sevoflurane anesthesia can be safely administered to these patients without adverse effects.[7] It has been suggested that continuation of beta-blocker therapy during the perioperative period may protect against TdP development that is related to an induced prolonged QT interval; however, there are case reports developing TdP, patients with LQTS may be more susceptible than different genotypes to arrhythmias induced by volatile agents; therefore, volatile agents should either be avoided altogether or used with great caution in these patients.[8] In this case, we gave the patient a very low dose of sevoflurane before opening the vascular access and stopped the sevoflurane after induction with propofol. Lidocaine was administered before intubation to take advantage of its arrhythmogenic effect. During the surgery, preparation stage, esmolol, magnesium, and the defibrillator were prepared for the treatment of TdP.

We did not administer any premedication before the surgery, but we administered 120 mg paracetamol and 15 mg phenobarbital (Paranox) rectally to prevent pain-related agitation during the postoperative period. During surgery, care was taken not to increase the Valsalva maneuver and ventilator pressures.

Patients with congenital LQTS are at increased risk for developing lethal arrhythmias associated with surgery, and this risk is greatest during periods of increased sympathetic stimulation (i.e., laryngoscopy and emergence); however, they can be safely managed with the appropriate preparation, attention to and selection of intra- and postoperative management, and close monitoring. The control of symptoms before surgery and continued use of a beta-blocker is fundamental to safe perioperative care. Close hemodynamic monitoring, avoidance of any drugs that prolong the QT interval, maintaining adequate anesthesia and adequate analgesics, normocarbia, normothermia, and normovolemia are important considerations for intra- and postoperative care. Halogenated volatile anesthetics known to prolong the corrected QT interval, also succinylcholine, pancuronium, neostigmine, atropine, and adrenalin, should be avoided.[9],[10] In our case, rocuronium, sugammadex, propofol, and remifentanil were administered as safe anesthetics. New studies are suggesting the use of propofol under the age of 3.[11] Propofol might sometimes be considered a desirable to overcome clinical dilemmas in pediatric anesthesia, especially life-threatening situations such as this case.

   Conclusion Top

Anesthesiologists must be prepared to recognize and promptly treat tachycardia and TdP, and arrhythmias should they occur during surgery on patients with LQTS. Anesthesia can be safely administered by taking precautions against perioperative complications. Children with sensorineural hearing loss should be alert for long QT syndrome perioperatively.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, et al. Prevalence of the congenital long-QT syndrome. Circulation 2009;120:1761-7.  Back to cited text no. 1
Schwartz PJ, Ackerman MJ, George AL Jr, Wilde AAM. Impact of genetics on the clinical management of channelopathies. J Am Coll Cardiol 2013;62:169-80.  Back to cited text no. 2
Priori SG, Schwartz PJ, Napolitano C, Bloise R, Ronchetti E, Grillo M, et al. Risk stratification in the long-QT syndrome. N Engl J Med 2003;348:1866-74.  Back to cited text no. 3
Schwartz PJ. Idiopathic long QT syndrome: Progress and questions. Am Heart J 1985;109:399-411.  Back to cited text no. 4
Staudt GE, Watkins SC. Anesthetic considerations for pediatric patients with congenital long QT syndrome. J Cardiothorac Vasc Anesth 2019;33:2030-8.  Back to cited text no. 5
Wallace E, Howard L, Liu M, O'Brien T, Ward D, Shen S, et al. Long QT syndrome: genetics and future perspective. Pediatr Cardiol 2019;40:1419-30.  Back to cited text no. 6
Whyte SD, Nathan A, Myers D, Watkins SC, Kannankeril PJ, Etheridge SP, et al. The safety of modern anesthesia for children with long QT syndrome. Anesth Analg 2014;119:932-8.  Back to cited text no. 7
Kumakura M, Hara K, Sata T. Sevoflurane-associated torsade de pointes in a patient with congenital long QT syndrome genotype 2. J Clin Anesth 2016;33:81-5.  Back to cited text no. 8
Kies SJ, Pabelick CM, Hurley HA, White RD, Ackerman MJ. Anesthesia for patients with congenital long QT syndrome. Anesthesiology 2005;102:204-10.  Back to cited text no. 9
Pleym H, Bathen J, Spigset O, Gisvold SE. Ventricular fibrillation related to reversal of the neuromuscular blockade in a patient with long QT syndrome. Acta Anaesthesiol Scand 1999;43:352-5.  Back to cited text no. 10
Kang P, Jang YE, Kim EH, Lee JH, Kim JT, Kim HS. Safety and efficacy of propofol anesthesia for pediatric target-controlled infusion in children below 3 years of age: A retrospective observational study. Expert Opin Drug Saf 2018;17:983-9.  Back to cited text no. 11


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