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CASE REPORT
Year : 2022  |  Volume : 25  |  Issue : 7  |  Page : 1196-1198

Post native uvulo-tonsillectomy hemorrhage as a cause of acquired long QT syndrome in a Nigerian girl: A case report


Department of Paediatrics, University of Uyo Teaching Hospital, Uyo, Nigeria

Date of Submission17-Nov-2021
Date of Acceptance23-May-2022
Date of Web Publication20-Jul-2022

Correspondence Address:
Dr. K Bassey
Department of Paediatrics, University of Uyo Teaching Hospital, PMB 1136, Uyo
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_1941_21

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   Abstract 


Long QT syndrome (LQTS) is a rare disease entity which until recently was not readily recognized as one of the causes of sudden cardiac death in children. It is a syndrome which can be congenital or acquired and is characterized by the prolongation of the QTc interval, the presence of some electrocardiographic abnormalities and other clinical parameters together with suggestive or definitive family history (Schwartz criteria). The index case is a 4-year-old female who initially presented for management on account of post native tonsillectomy hemorrhage with secondary severe anemia and associated sepsis who subsequently developed bradycardia and marked prolongation of the QTc interval on electrocardiogram. Possible factors implicated as a cause of the prolongation of the QTc include severe anemia and anesthetic drugs with a probability of an underlying genetic cause. This case highlights a rare cause of sudden cardiac death in children in our environment with the attendant difficulties in making a genetic diagnosis due to inadequate laboratory facilities.

Keywords: Female, hemorrhage, long QTc, syndrome, tonsillectomy


How to cite this article:
Okpokowuruk F S, Bassey K. Post native uvulo-tonsillectomy hemorrhage as a cause of acquired long QT syndrome in a Nigerian girl: A case report. Niger J Clin Pract 2022;25:1196-8

How to cite this URL:
Okpokowuruk F S, Bassey K. Post native uvulo-tonsillectomy hemorrhage as a cause of acquired long QT syndrome in a Nigerian girl: A case report. Niger J Clin Pract [serial online] 2022 [cited 2022 Aug 8];25:1196-8. Available from: https://www.njcponline.com/text.asp?2022/25/7/1196/351466




   Introduction Top


The Long QT Syndrome (LQTS) is a hereditary disorder where affected individuals have delayed ventricular repolarization evidenced by QT prolongation on the electrocardiogram.[1] It is a relatively rare disorder characterized by syncope, seizures, and death,[2] affecting 1:5000 individuals with a variable phenotype ranging from asymptomatic electrocardiographic changes to sudden death.[3] LQTS is due to a monogenic mutation in myocyte-ion channels and over 600 mutations have been identified so far.[3] It is principally inherited as an autosomal dominant condition with variable penetrance.[2],[3] About 85% of known cases are inherited from one or both parents while 15% arise from spontaneous mutations.[2] Specific disorders such as the Jervell and Lange-Nielsen Syndrome associated with hearing loss and the Romano-Ward Syndrome with a milder phenotypic course have been specifically identified and characterized based on their specific ion-channel mutations.[2],[4] The risk of sudden cardiac death resulting from the development of polymorphic ventricular tachycardia (Torsade de pointes) is the major clinical complication of LQTS.

The definitive diagnosis of LQTS can be challenging in Paediatric patients due to the failure of many physicians to recognize the syndrome[5] and the possibility of other causes of exercise-induced syncope in children such as hypertrophic cardiomyopathy and anomalous left coronary artery arising from the pulmonary artery (ALCAPA).[2] It is also known that some children with LQTS have normal resting electrocardiograms and require a brisk standing test to induce changes in the QTc.[6] Scoring systems such as the Schwartz[7] and Keating Scores[8] have been developed to aid in the diagnosis but have been found to have exceptional specificity but poor sensitivity.[8] The length of the QTc interval remains the most sensitive screening tool for LQTS and a genetic test is done to confirm mutations in genotyped families.[8]

We present the case of a 4-year-old girl who presented with features of LQTS following an examination under anesthesia (EUA) and tonsillectomy done to arrest hemorrhage from the tonsillar bed arising from an attempted native tonsillectomy.


   Case History Top


A 4 year 10-month-old girl who was referred for a cardiology consult on account of persistent post-operative bradycardia noticed about 30 hours post-examination under anesthesia and tonsillectomy. Her heart rate ranged from 42 to 54 beats per minute. There was no recent history of use or administration of parasympathomimetic agents or adrenergic blockers.

She was admitted two days earlier on account of fever of five days duration, vomiting, and passage of blood in stool of six hours duration. Child had been taken to a traditional healer who attempted a tonsillectomy (? instrument used) and child developed hematemesis thereafter with over 15 episodes of vomiting of bright red blood, about 50 ml per episode. Child also had multiple episodes of passage of frank blood in stool.

Initial physical examination on admission revealed an acutely ill, dyspnoeic. and severely pale child, afebrile (T = 36.4°C) with bilateral submandibular swelling.

Cardiovascular examination showed a pulse rate of 110/min, regular, displaced apex and S1, S2, S3 + gallop rhythm, patient was dyspnoeic with respiratory rate of 52/min. Significant findings in the digestive system included drooling of frank blood and soft, tender hepatomegaly of 4 cm below the right costal margin.

Urgent packed cell volume (PCV) on admission was 11%. Prior to transfusion, her genotype was AA. She was subsequently transfused with 360 ml of fresh whole blood. Post-transfusion PCV - 30%, Total white blood cell count (TWBC) - 20.5 × 10^9/L, Neutrophils - 52% with neutrophilic left shift. Findings on EUA showed bilateral bleeding and inflamed tonsils and oropharynx for which patient had Tonsillectomy carried out by the ENT team under general anesthesia.

ECG done showed bradycardia with sinus arrythmia, normal P wave, normal QRS axis. QTc was markedly prolonged at 508 ms; patient also met voltage criteria for left ventricular hypertrophy [Figure 1]. Serum E/U/Cr: K+ ranged between 3.5-4.5 mmol/l, other electrolytes were essentially normal. Serum magnesium and calcium was not done. Further evaluation revealed a history of sudden cardiac death during pregnancy in a paternal aunt. Her modified Schwartz score for long QT syndrome was 4.
Figure 1: Electrocardiogram of patient BO showing sinus arrhythmia, prolonged PR interval, prolonged QTc interval, and voltage criteria for left ventricular hypertrophy

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The patient was subsequently managed for acquired long QT syndrome r/o congenital long QT syndrome following post native tonsillectomy hemorrhage and sepsis. She was treated with intravenous Ceftriaxzone, Genticin, and oral Propranolol. She was discharged and followed up in clinic over a period of one year with repeat ECGs (five in number) which showed gradual reduction in QTc from 508 to 440 ms. The latest ECG done exactly one year after showed a borderline QTc of 440 ms in lead II. Four post-transfusion PCVs' were also carried out which ranged between 24 and 30%.


   Discussion Top


LQTS is a rare disease entity which until recently was not readily recognized as one of the causes of sudden cardiac death in children. Long QT syndrome can be congenital or acquired.

In the index patient, her initial QTc interval was markedly prolonged and was associated with significant bradycardia and a history of sudden death during pregnancy in her paternal aunt aged below 30 years. Based on the modified Schwartz criteria,[9] this patient had a total score of 4 which indicates a high probability of LQTS.

LQTS as a disease entity has been poorly elucidated in our environment as there has been only one published report in children on this condition with Onalo and Babaniyi highlighting the difficulties in making a diagnosis of LQTS in resource poor environments such as ours.[10] Prevalence of prolonged QTc interval in this environment is low with 1.8% of a study population of adolescents on routine ECG screening demonstrating this abnormality.[11]

A combination of congenital and acquired factors could be implicated in this patient's clinical condition. A positive history of sudden cardiac death in pregnancy in the patient's paternal aunt gives rise to a suspicion of LQTS type 2 although this could not be confirmed because of the absence of facilities for genetic testing.[12] Acquired causes of LQTS are far more common than congenital causes with most of the acquired causes resulting from adverse effects of drugs including anesthetic drugs or electrolyte abnormalities, which can interact with the gene related to LQTS2.[13] The anesthetic drug used for this patient was Isoflurane which although it has been found to prolong QTc interval by some researchers, it has also been safely used in those with LQTS.[14] In the index patient, no pre-operative ECG was carried out as patient came in as an emergency. Also no electrolyte abnormalities were detected although serum calcium and magnesium were not done as the diagnosis of ALQTS was retrospective when patient developed bradycardia post-surgery. Myocardial ischaemia is another factor that has been implicated as a cause of acquired LQTS.[13] The patient under review was severely anemic arising from massive hemorrhage post attempted native uvulo-tonsillectomy. Prolongation of the QTc interval from anemia has been demonstrated by some researchers in our environment[15],[16] although Fei in his conclusions posited that anemia does not directly cause QTc prolongation however, myocardial anemia as a probable cause of LQTS in this patient cannot be ruled out.[17]

In conclusion, routine follow-up of this patient in clinic over a period of one year revealed a gradual reduction in the QTc interval to 440 ms [lead II - [Figure 2]] which is the borderline cutoff for normal indicating that this was likely a case of acquired LQTS although an underlying congenital syndrome still cannot be ruled out. The availability of facilities to conduct genetic testing in these patients remains a major challenge in the diagnosis and prevention of sudden cardiac death arising from LQTS in our environment. This calls for an improvement in the diagnostic capabilities of our healthcare facilities while clinicians need to have a high index of suspicion.
Figure 2: Electrocardiogram of patient BO one year after showing prolonged PR interval, borderline QTc

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Goldenberg I, Zareba W, Moss AJ. Long QT syndrome. Curr Probl Cardiol 2008;33:629-94.  Back to cited text no. 1
    
2.
Garson Jr A, Dick M, Fournier A, Gillette PC, Hamilton R, Kugler JD, et al. The long QT syndrome in children. An international study of 287 patients. Circulation 1993;87:1866-72.  Back to cited text no. 2
    
3.
Etheridge SP, Sanatani S, Cohen MI, Albaro CA, Saarel EV, Bradley DJ. Long QT syndrome in children in the era of implantable defibrillators. J Am Coll Cardiol 2007;50:1335-40.  Back to cited text no. 3
    
4.
Splawski I, Timothy KW, Vincent GM, Atkinson DL, Keating MT. Molecular basis of the long-QT syndrome associated with deafness. N Engl J Med 1997;336:1562-7.  Back to cited text no. 4
    
5.
Viskin S, Rosovski U, Sands AJ, Chen E, Kistler PM, Kalman JM, et al. Inaccurate electrocardiographic interpretation of long QT: The majority of physicians cannot recognize a long QT when they see one. Heart Rhythm 2005;2:569-74.  Back to cited text no. 5
    
6.
Filippini LH, Postema PG, Zoubin K, Hermans BJ, Blom NA, Delhaas T, et al. The brisk-standing-test for long QT syndrome in prepubertal school children: Defining normal. EP Europace 2018;20:108-12.  Back to cited text no. 6
    
7.
Schwartz PJ. The idiopathic long qt syndrome: Progress and questions. Am Heart J 1985;109:399-411.  Back to cited text no. 7
    
8.
Hofman N, Wilde AA, Kääb S, van Langen IM, Tanck MW, Mannens MM, et al. Diagnostic criteria for congenital long QT syndrome in the era of molecular genetics: Do we need a scoring system? Eur Heart J 2007;28:575-80.  Back to cited text no. 8
    
9.
Sharma N, Cortez D, Disori K, Imundo JR, Beck M. A review of long QT syndrome: Everything a hospitalist should know. Hosp Pediatr 2020;10:369-75.  Back to cited text no. 9
    
10.
Onalo R, Babaniyi IB. Congenital long-QT syndrome in a Nigerian child: A case report. Niger Postgrad Med J 2013;20:237-40.  Back to cited text no. 10
  [Full text]  
11.
Tabansi PN, Dibua O. Screening for arrhythmias in secondary school students in Port Harcourt Nigeria. Niger J Paediatr 2019;46:61-7.  Back to cited text no. 11
    
12.
Seslar SP, Zimetbaum PJ, Berul CI, Josephson ME. Clinical features of congenital long QT syndrome. Available from: https://somepomed.org/articulos/contents/mobipreview.htm?8/62/9185?source=see_link#H18. [Last accessed on 2021 Jan 26].  Back to cited text no. 12
    
13.
O'Hare M, Maldonado Y, Munro J, Ackerman MJ, Ramakrishna H, Sorajja D. Perioperative management of patients with congenital or acquired disorders of the QT interval. Br J Anaesth 2018;120:629-44.  Back to cited text no. 13
    
14.
Fazio G. Drugs to be avoided in patients with long QT syndrome: Focus on the anaesthesiological management. World J Cardiol 2013;5:87-9.  Back to cited text no. 14
    
15.
Sadoh W, Uduebor J. Electrocardiographic changes and troponin T levels in children with severe malaria anemia and heart failure. Niger J Clin Pract 2017;20:552-6.  Back to cited text no. 15
[PUBMED]  [Full text]  
16.
Bode-Thomas F, Ogunkunle OO, Omotoso AB. Cardiac arrythmias in children with sickle cell anaemia. Niger J Paediatr 2003;30:13-7.  Back to cited text no. 16
    
17.
Fei Y, Li Y, Surkis W, Zhang L. Does anemia cause QT prolongation in patients with hematologic disorders? Chin Med J 2015;128:3385-6.  Back to cited text no. 17
[PUBMED]  [Full text]  


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