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Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 97-98

Cardiac arrest secondary to Jervell-Lange-Neilson syndrome

Department of Pediatrics, Advanced Pediatric Critical Care Centre, Wanless Hospital, Miraj, Maharashtra, India

Date of Submission15-Feb-2020
Date of Decision28-Feb-2020
Date of Acceptance07-Mar-2020
Date of Web Publication10-Apr-2020

Correspondence Address:
Dr. Priya Sakte
Department of Pediatrics, Advanced Pediatric Critical Care Centre, Wanless Hospital, Miraj, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JPCC.JPCC_32_20

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How to cite this article:
Sakte P, Antin J, Jangam S, Patki V. Cardiac arrest secondary to Jervell-Lange-Neilson syndrome. J Pediatr Crit Care 2020;7:97-8

How to cite this URL:
Sakte P, Antin J, Jangam S, Patki V. Cardiac arrest secondary to Jervell-Lange-Neilson syndrome. J Pediatr Crit Care [serial online] 2020 [cited 2020 May 31];7:97-8. Available from: http://www.jpcc.org.in/text.asp?2020/7/2/97/282223

A 6-year-old female child, with a known history of congenital severe bilateral sensory neural deafness, was taken for cochlear implant surgery. She presented with sudden cardiac arrest during the induction of general anesthesia. She was transferred to the Advanced Pediatric Critical Care Centre, Wanless Hospital, Miraj. There was no prior history suggestive of syncopal attacks. She was not on any medications before her hospitalization. There was no delay in the attainment of motor and social milestones. Congenital sensorineural deafness is present in two older female siblings but no history of syncopal attacks or sudden death in any family members.

Postresuscitation, she was hemodynamically stable on ventilator and adrenaline infusion. There were no significant findings on physical examination revealing the cause for the cardiac arrest. Laboratory findings and investigations revealed normal blood count and biochemistry. Chest radiograph showed normal lung fields and cardiac size. Electrocardiogram (ECG) revealed long QTc interval (0.62 s) [Figure 1].
Figure 1: Electrocardiograph long lead II showing long QTc interval

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Two-dimensional echo (postcardiac arrest) showed trivial TR, mild right ventricular dysfunction, left ventricular ejection fraction 55%, and structurally normal heart.

Findings of long QT interval combined with congenital sensorineural hearing impairment led to the clinical diagnosis of Jervell and Lange-Neilson syndrome, one of the rarer types of long QT syndromes (LQTSs). The child was started on oral beta-blocker metoprolol (2 mg/kg/day). She was weaned off ventilator by 24 h of hospitalization and was discharged in a stable condition on day 4. She continues to be on treatment with oral metoprolol and has had no further cardiac events post-discharge from the hospital.

LQTSs are genetic abnormalities of ventricular repolarization, with an estimated incidence of about 1 per 10,000 birth. They present as a long QTc interval on ECG (>0.47 s) and are associated with malignant ventricular arrhythmias (torsades de pointes and ventricular fibrillation). They are a cause of syncope and sudden death.[1] Jervell-Lange-Neilson syndrome (JLNS) is an autosomal recessive disorder characterized by congenital sensorineural deafness and long QT interval on ECG. It is associated with the genes KCNQ1 (JLNS type 1) and KCNE1 (JLNS type 2).

Clinical manifestation of LQTS in children is most often a syncopal episode brought on by exercise, fright, or sudden startle. They may present with seizures, presyncope, or palpitations. Approximately 10% are initially in cardiac arrest.[2] In our case, cardiac arrest was the initial presentation.

The diagnosis is based on the ECG and clinical criteria. A heart rate-corrected QT interval of >0.47 s is highly indicative, whereas a QT interval of >0.44 s is suggestive. Other features include notched T-waves in 3 leads, T-wave alternans, a low heart rate for age, a history of syncope (especially with stress), and a familial history of either LQTS or sudden death. Genotyping can identify the mutation in approximately 80% of patients known to have LQTS by clinical criteria.[1]

Treatment of LQTS includes the use of beta-blocking agents at doses that blunt the heart rate response to exercise. Some patients may require a pacemaker because of drug-induced bradycardia. In patients with continued syncope, despite treatment, an implantable cardiac defibrillator is indicated for those who do not respond to beta-blockers and those who have had a cardiac arrest.[1]

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

van Hare GF. Long Q-T syndromes. In: Kleigman, Stanton, St Gene, Schor, editors. Nelson Textbook of Pediatrics. 1st South-East Asia ed., Vol. 2, Ch. 435. Elsevier India; 2015. p. 2258-9.   Back to cited text no. 1
Schwartz PJ, Spazzolini C, Crotti L, Bathen J, Amlie JP, Timothy K, et al. The Jervell and Lange-Nielsen syndrome: Natural history, molecular basis, and clinical outcome. Circulation 2006;113:783-90.  Back to cited text no. 2


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