PREVALENCE OF LONG QT SYNDROME AND OTHER CARDIAC DEFECTS IN DEAF-MUTE CHILDREN
Abstract
Background: Long QT syndrome is considered a fatal disease because of its association withventricular arrhythmias and sudden cardiac death. Objectives of study were to determine the prevalenceof long QT syndrome and other heart diseases, in deaf-mute children. Methods: A Cross-sectionaldescriptive study was conducted at Cholistan special education centre and Cardiology department,Sheikh Zayed hospital Rahim Yar Khan, Pakistan in September 2006. A total of 104 congenitally deafmute children were assessed. Height, weight and blood pressure measured, 12-lead electrocardiogramdone and QTc calculated using Bazette’s formula. Children with prolonged QTc underwent 24-hourambulatory ECG recording. All were auscultated following complete protocol. A child with murmurwas further evaluated with colour Doppler echocardiography. Audiometry was performed on all thechildren and the result interpreted according to WHO recommendations. Diagnosis of LQTS was basedon Revised Schwartz criteria. Results: Out of 104 children, 62 were male with mean age 11.89 yrs. Theaverage systolic and diastolic BP was 97/67 mmHg. Average height was 126 Cm. All children hadmoderate to severe bilateral sensorineural hearing loss (40–80 dB). One child had associated PatentDuctus Arteriosis. Fifteen had an innocent murmur. Prevalence of congenital heart disease was found tobe 0.1/1000. Four children had QT interval more than 440 mSec, (range 0.46–0.47 mSec.). Bothgenders were equally affected. Three children had high probability of LQTS and one had intermediateprobability. Screening of family of these 4 patients showed prolonged QT interval in the sibling of onepatient. Conclusion: Our study highlights the significant prevalence of Jervell Lange-NielsenSyndrome in Pakistani deaf-mute children, which may be associated to the high level of consanguinityin this region. Awareness of this syndrome among health care providers is needed as timely diagnosisand subsequent treatment may prevent fatal complications.Keywords: Long QT Syndrome, Deafness, Congenital Heart DefectsReferences
Jervell A, Lange-Nielsen F. Congenital deaf-mutism, functional
heart disease with prolongation of the QT interval and sudden
death. Am Heart J 1957;54:59–68
James TN. Congenital deafness and cardiac arrhythmias. Am J
Cardiol 1967;19:627–30
Morita H, Wu J, Zipes DP. The QT syndromes: long and
short. Lancet 2008;372:750–63
Pakistan Demographics Profile. Available at: http://www.
indexmundi.com/pakistan/demographics_profile.html
Sajjad M, Khattak AA, Bunn JE, Mackenzie I. Causes of
childhood deafness in Pukhtoonkhwa Province of Pakistan and
the role of consanguinity. J Laryngol Otol 2008;122:1057–63.
Bazett HC. An analysis of the time-relations of the
electrocardiagram. Heart 1920;7:353-370
Schwartz PJ, Moss AJ, Vincent GM, Crampton RS: Diagnostic
criteria for the long QT syndrome. An update. Circulation
;88:782-784
Dhingra PL. Hearing loss. In: Diseases of Ear, Nose and Throat.
New Delhi: Elsevier; 2005:38–51.
Fraser GR, Froggatt P, James TN. Congenital deafness associated
with electrocardiographic abnormalities, fainting attacks and
sudden death: a recessive syndrome. QJ Med 1964;33:361–85.
Sanchez-Cascos A, Sanchez- Harguindey L, DeRabago P.
Cardio-auditory syndrome; cardiac and genetic study of 511
deaf-mute children. Br Heart J 1969;31:26–30.
Jay FE, Olley PM, Partington MW, Kavety VB, Ahmad G.
Surdo-cardiac syndrome: Incidence among children in schools
for the deaf. Can Med Assoc J 1971;105:718–20.
J Ayub Med Coll Abbottabad 2011;23(1)
http://www.ayubmed.edu.pk/JAMC/23-1/Ahsan.pdf
Ocal B, Imamoglu A, Atalay S, Ercan Tutar H. Prevalence of
idiopathic long QT syndrome in children with congenital
deafness. Pediatr Cardiol 1997;18:401–5.
Sopontammarak S, Khongphatthanayothin A, Sa-Nguanchua P.
Prevalence of idiopathic long QT syndrome in congenital
sensori-neural hearing loss students of Songkhla School for the
Deaf. J Med Assoc Thai 2003;86:1149–55
Yuen LK, Lam CW, Fong NC, Tang PM, Shek CC, Chan
YW, et al. Jervell-Lange Nielsen syndrome in a Pakistani
family.Hong Kong Med J 2004;10:351–4.
Csanády M, Hogye M, Forster T. Hypertrophic cardiomyopathy
associated with congenital deaf-mutism. Eur Heart
J 1987;8(5):528–34.
Mohiddin SA, Ahmed ZM, Griffith AJ, Tripodi D, Friedman
TB, Fananapazir L, et al. Novel association of hypertrophic
cardiomyopathy, sensorineural deafness, and a mutation in
unconventional myosin VI (MYO6). J Med
Genet 2004;41:309–14.
Schönberger J, Levy H, Grünig E, Sangwatanaroj S, Fatkin D,
MacRae C, et al. Dilated Cardiomyopathy and Sensorineural
Hearing Loss: A Heritable Syndrome That Maps to 6q23–24:
Circulation 2000;101:1812–8.
Uçar T, Tutar E, Tekin M, Atalay S. Heart rate variability in
children with congenital sensorineural deafness. Turk J
Pediatr 2010;52(2):173–8.
Priori SG, Napolitano C, Schwartz PJ. Association of long QT
syndrome loci and cardiac events among patients treated with
beta-blockers. JAMA 2004;292:1341–4.
Siem G, Früh A, Leren TP, Heimdal K, Teig E, Harris S. Jervell
and Lange-Nielsen syndrome in Norwegian children: aspects
around cochlear implantation, hearing, and balance. Ear
Hear 2008;29(2):261–9.
Downloads
Published
Issue
Section
License
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.