Malignant familial long QT syndrome.

Malignant familial long QT syndrome. Research Abstract Details 

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Malignant familial long QT syndrome. Abstract Text:

We report a family with congenital long QT syndrome, an inherited disorder of myocardial repolarization in which affected individuals have prolongation of corrected QT interval on the electrocardiogram and a tendency to develop ventricular arrhythmia, leading to syncope, convulsion or sudden death. Our family is characterized by several affected members (11/16), early onset of symptoms, malignant course prior to diagnosis and good response to beta-blocker therapy. The genetic basis of long QT syndrome has been traced to defective proteins encoding cardiac ion channels. Diagnosis is based on an unexplained prolongation of QT interval >0.45 second in the presence of suggestive symptoms or evidence, or both of a familial pattern. Beta-adrenergic blocker therapy gives symptomatic relief in 80%-85% of patients. Precipitating factors like vigorous exercise especially swimming and exposure to significant emotional or auditory stimuli should be avoided. Occasional patients require in addition, a demand cardiac pacemaker, left cardiac sympathectomy or an implantable cardioverter-defibrillator, or both. Regular follow up is mandatory even after subsidence of symptoms.

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Malignant familial long QT syndrome. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Saudi medical journal

VOLUME: 23

Page Numbers: 738-42

Journal Abbreviation: Saudi Med J

ISSN: 0379-5284

DAY: 23

MONTH: Jun

YEAR: 2002

Malignant familial long QT syndrome. Information

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LANGUAGE: eng

NlmUniqueID: 7909441

Malignant familial long QT syndrome. Keywords Mesh Terms:

KEYWORDS: Propranolol

MESH TERMS: therapeutic use

Chemical & Substance for Abstract: Malignant familial long QT syndrome. Information

Substance Name: Propranolol

Registry Number: 525-66-6

Grant and Affiliation Information for Malignant familial long QT syndrome.

AFFILIATION: Department of Pediatric Cardiology, Royal Hospital, Muscat, Sulanate of Oman. ragusha@omantel.net.om

Country: Saudi Arabia

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MEDLINETA: Saudi Med J

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