ELECTROCARDIOGRAPHIC MANIFESTATIONS IN PAEDIATRIC WILSON DISEASE

Authors

  • Sara Batool Hamdani COLLEGE OF PHYSICIANS &SURGEONS PAKISTAN
  • Huma Arshad Cheema children hospital lahore
  • Anjum Saeed children hospital lahore
  • Hassan Suleman Malik children hospital lahore
  • Tayyaba Sehar rawalpindi institute of cardiology

Abstract

Background: Wilson disease (WD) is one of the most common metabolic liver diseases in older children. It has a strong genetic background with autosomal recessive inheritance. WD is a multisystem disorder with predominant hepatic and neurological manifestations and variable age of presentation. The data on cardiac manifestations in children is very limited and only few adult studies are available in the literature. This study was planned to determine the frequency and spectrum of Electrocardiographic (ECG) changes in pediatric WD. Methods: This was an observational cross-sectional study conducted at The Children Hospital & the Institute of Child Health, Lahore, from January 2015 to January 2017. The children diagnosed as Wilson disease were enrolled for the recording of resting ECG. The ECG changes were seen and discussed with an experienced pediatric cardiologist who was involved and explained about the objectives of study. Result: Total 55 patients were enrolled but record of ECG was missing for 4 patients and excluded from the study.  Out of 51 patients 22 had at least one ECG abnormality. Most frequent findings seen were T wave abnormality in 18 patients (35.2%) followed by sinus tachycardia and sinus bradycardia in 12 and 8 patients respectively. Other abnormalities included bifid P waves, ST segment changes each of 2 patients, and one premature ventricular contraction. QRS details including axis, complex, amplitude ratio and QT interval was normal in all the patients. There was no mortality during the study period due to cardiac cause. Conclusion: ECG abnormalities are not uncommon in pediatric WD but of mild nature. These are presumably related to underlying cardiomyopathy due to deposition of copper in heart which can be quantified by cardiac magnetic resonance imaging (MRI) and echocardiography is required to confirm ECG abnormalities detected.Keyword: Wilson disease; ECG changes; cardiomyopathy

Author Biographies

Sara Batool Hamdani, COLLEGE OF PHYSICIANS &SURGEONS PAKISTAN

senior registrar pediatric gastroenterology ,hepatology & nutrition

Huma Arshad Cheema, children hospital lahore

head of deartment pediatric gastroenterology &heaptologychildren hospital lahore

Anjum Saeed, children hospital lahore

associate prfessor pediatric gastroenterology & heaptologychildren hospital lahore

Hassan Suleman Malik, children hospital lahore

associate professor pediatric gastroenterology & hepatologychildren hospital lahore

Tayyaba Sehar, rawalpindi institute of cardiology

senior registrar rawalpindi institute of cardiology

References

Roberts EA, Schilky ML. Diagnosis and treatment of Wilson’s disease. An update. Hepatology 2008;47(6):2089–111.

Rosencrantz R, Schilsky M. Wilson’s disease: pathogenesis and clinical considerations in diagnosis and treatment. Semin Liver Dis 2011;31(3):245–59.

Bennett J, Hahn SH. Clinical molecular diagnosis of Wilson’s disease. Semin Liver Dis 2011;13(3):233–8.

Seo JK. Wilson disease: an update. Korean J hepatol 2006;12(3):333–63.

Rosencrantz, R. and M. Schilsky. Wilson disease: Pathogenesis and clinical considerations in diagnosis and treatment. Semin Liver Dis 2011;31 245–59.

Taly AB, Prashanth Lk, Sinha S. Wilson’s disease: An Indian perspective. Neurol India 2009;57(5):528–40.

Samiullah S, Salma S, Faheemullah S, Iftikhar K. Wilson’s disease; Various shapes of one disease. Pak J Med Sci 2010;26(1):158–16.

Ala A, Walker P, Ashkan K, Dooley SJ, Schilsky ML. Wilson’s Disease. Lancet 2007;369(9559):39–408.

KaunP. Cardiac wilson’s disease. Chest 1987;91(4):579–83.

Meenakshi-Sundaram S, Taly AB, Kamath V, Arunodaya GR, Rao S, Swamy HS. Autonomic dysfunction in Wilson disease-a clinical and electrophysiological study. Clin Auton Res 2002;12(3):185–9.

Chu EC, Chu NS, Huang CC. Autonomic involvement in Wilson’s disease, a study sympathetic response and RR variation. J Neurol Sci 1997;149(2):131–7.

Meenakshi-Sundaram S, Sinha S, Rao M, Prashanth LK, Arunodaya GR, Rao S, et al. Cardiac involvement in Wilson’s disease-an electrocardiographic observation. J Assoc Physician India 2004;52:59–6.

Bajaj BK, Wadhwa A, Singh R, Gupta S. Cardiac arrhythmia in Wilson’s disease: An oversight and overlooked entity. J Neuro Sci Rural Pract 2016;7(4):587–9.

Karakurt C, Celik S, Selmoqlu A, Varol I, Karabiber H, Yologlu S. Strain and strain rate echocardiography in children with Wilson’s disease. Cardiovasc J Afr 2016;27(5):307–14.

Meenakshi-Sundaram S, Mahadevan A, Taly A, Arunodaya G, Swamy H, Shankar S. Pathology of Wilson’s disease revisited. Ann Indian Acad Neurol 2003;6:67.

Prashanth LK, Taly AB, Sinhas S, Arunodaya GR, Swamy HS. Wilson's disease: Diagnostic errors and clinical implications. J Neurol Neurosurg Psychiatry 2004;75(6):907–9.

Hlubocka Z, Marecek Z, Linhart A, Kejkova E, Pospisilova L, Martasek P, et al. Cardiac involvement in Wilson disease. J Inherit Metab Dis 2002;25(4):269–77.

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Published

2017-12-27

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