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


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


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