The congenital long QT syndrome

被引：4

作者：

Shanbag P. ^{[1
,2
]}

Govindakumar P.T. ^{[1
]}

Vaidya M. ^{[1
]}

Joshi V. ^{[1
]}

Shahid S.K. ^{[1
]}

机构：

[1] Pediatric Intensive Care Unit (PICU), Department of Pediatrics, Lokmanya Tilak Munic. Med. Coll. G., Sion, Mumbai

[2] C-16 Shanti CHS, Mahim, Mumbai-400 016, Mogal Lane

来源：

The Indian Journal of Pediatrics | 2002年 / 69卷 / 2期

关键词：

Congenital long QT syndrome; Heart block;

D O I：

10.1007/BF02859375

中图分类号：

学科分类号：

摘要：

Objective : The long QT syndrome (LQTS) is a disorder of the electrical system of the heart, due to dysfunction of the ion channels and involving the repolarisation process. The inherited form occurs when there is a mutation in one of the genes which encode the making of a channel. Prolongation of the QT interval renders the patient vulnerable to an arrythmia called torsade de pointes, resulting in syncope and sudden death. Methods : Three children with the congenital long QT syndrome presented to the pediatric department, one of them also having a 2:1 atrio-ventricular block. The parents and siblings of these children were screened for the long QT syndrome with an electrocardiogram. 2D echocardiography was done to rule out structural abnormalities and audiometry for deafness. Results : Four family members were identified on screening to have LQTS. Propranolol was started on all children with LQTS. The child with heart block also received a pacemaker. LQTS must be considered in all patients presenting with syncope especially if associated with deafness and/or a family history of sudden deaths in infancy or childhood. Conclusion : The corrected QT interval must be determined in all children with heart block since the two conditions are often associated.

引用

页码：141 / 144

页数：3

共 13 条

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