Niaz Ahsan, Rizvi Syed Faiz-ul-Hassan, Khurram Daniyeh
Department of Cardiology, Sheikh Zayed Medical College/Hospital, Rahim Yar Khan, Pakistan.
J Ayub Med Coll Abbottabad. 2011 Jan-Mar;23(1):5-8.
Long QT syndrome is considered a fatal disease because of its association with ventricular arrhythmias and sudden cardiac death. Objectives of study were to determine the prevalence of long QT syndrome and other heart diseases, in deaf-mute children.
A Cross-sectional descriptive 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 deaf-mute children were assessed. Height, weight and blood pressure measured, 12-lead electrocardiogram done and QTc calculated using Bazette's formula. Children with prolonged QTc underwent 24-hour ambulatory ECG recording. All were auscultated following complete protocol. A child with murmur was further evaluated with colour Doppler echocardiography. Audiometry was performed on all the children and the result interpreted according to WHO recommendations. Diagnosis of LQTS was based on Revised Schwartz criteria.
Out of 104 children, 62 were male with mean age 11.89 yrs. The average systolic and diastolic BP was 97/67 mmHg. Average height was 126 Cm. All children had moderate to severe bilateral sensorineural hearing loss (40-80 dB). One child had associated Patent Ductus Arteriosis. Fifteen had an innocent murmur. Prevalence of congenital heart disease was found to be 0.1/1000. Four children had QT interval more than 440 mSec, (range 0.46-0.47 mSec.). Both genders were equally affected. Three children had high probability of LQTS and one had intermediate probability. Screening of family of these 4 patients showed prolonged QT interval in the sibling of one patient.
Our study highlights the significant prevalence of Jervell Lange-Nielsen Syndrome in Pakistani deaf-mute children, which may be associated to the high level of consanguinity in this region. Awareness of this syndrome among health care providers is needed as timely diagnosis and subsequent treatment may prevent fatal complications.
长QT综合征因其与室性心律失常和心源性猝死相关，被视为一种致命疾病。本研究的目的是确定聋哑儿童中长QT综合征及其他心脏病的患病率。
2006年9月在巴基斯坦拉希姆亚尔汗谢赫扎耶德医院的乔利斯坦特殊教育中心和心脏病科进行了一项横断面描述性研究。共评估了104名先天性聋哑儿童。测量身高、体重和血压，进行12导联心电图检查，并使用巴泽特公式计算QTc。QTc延长的儿童进行24小时动态心电图记录。按照完整方案对所有儿童进行听诊。有杂音的儿童进一步接受彩色多普勒超声心动图检查。对所有儿童进行听力测定，并根据世界卫生组织的建议解释结果。LQTS的诊断基于修订的施瓦茨标准。
104名儿童中，62名男性，平均年龄11.89岁。平均收缩压和舒张压为97/67 mmHg。平均身高为126厘米。所有儿童均有中度至重度双侧感音神经性听力损失（40 - 80分贝）。一名儿童伴有动脉导管未闭。15名儿童有生理性杂音。先天性心脏病的患病率为0.1/1000。4名儿童的QT间期超过440毫秒（范围为0.46 - 0.47毫秒）。男女受影响程度相同。3名儿童LQTS可能性高，1名儿童可能性中等。对这4名患者的家族筛查显示，1名患者的兄弟姐妹QT间期延长。
我们的研究突出了巴基斯坦聋哑儿童中杰韦尔-朗格-尼尔森综合征的显著患病率，这可能与该地区较高的近亲结婚率有关。医疗保健提供者需要了解这种综合征，因为及时诊断和后续治疗可能预防致命并发症。
