PREVALENCE OF LONG QT SYNDROME AND OTHER CARDIAC DEFECTS IN DEAF-MUTE CHILDREN

Authors

  • Ahsan Niaz
  • Syed Faiz-ul-hassan Rizvi
  • Daniyeh Khurram

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 Defects

References

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.

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Published

2011-03-01