Jervell and Lange-Nielsen syndrome is an autosomal recessive rare form of LQTS that affects between 1 in 1,000,000–4,000,000 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 Q–T 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 d 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 developing 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 mg/kg 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 torsades de point, 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 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, neostigmin, atropine, and adrenalin, should be avoided (9, 10). In our case, rocuronium, sugammadex, propofol, and remifentanil were administered as safe anesthetics.