| The long QT syndrome with impaired atrioventricular conduction: a malignant variant in infants. | |
| | |
MedLine Citation:
|
PMID: 9835268 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
INTRODUCTION: The long QT syndrome (LQTS) is occasionally complicated by impaired AV conduction, mostly 2:1 AV block. This form of LQTS can manifest before birth or during neonatal life, and it is more sporadic than familial. It is usually an isolated disorder, although it can be accompanied by a variety of cardiovascular and other anomalies. In spite of different treatment modes, mortality is high. METHODS AND RESULTS: The reported case presented not only with 2:1 AV conduction, but also with Wenckebach episodes with impaired right and left bundle branch conduction, and decremental conduction in the His-Purkinje axis. We also observed sinus pauses and accelerated AV junctional escape beats. CONCLUSION: Our findings, and similar observations by others, suggest involvement of the sinus node and the distal conduction system in this form of the LQTS. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed. Development of an internal cardiac defibrillator for this young age group is necessary. |
| | |
Authors:
|
A P Gorgels; F Al Fadley; L Zaman; M J Kantoch; Z Al Halees |
Related Documents
:
|
12521368 - Lymph node infarction. an immunohistochemical study of 11 cases. 1514488 - Safety and diagnostic accuracy of adenosine thallium-201 scintigraphy in patients unabl... 3711498 - Bundle branch reentrant tachycardia treated by electrical ablation of the right bundle ... 1191458 - Complete heart block and systemic lupus erythematosus. 7898088 - The renin-angiotensin system as a target for therapeutic intervention. 21108208 - St segment elevation myocardial infarction due to slow coronary flow occurring after ca... |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: Journal of cardiovascular electrophysiology Volume: 9 ISSN: 1045-3873 ISO Abbreviation: J. Cardiovasc. Electrophysiol. Publication Date: 1998 Nov |
Date Detail:
|
Created Date: 1999-02-10 Completed Date: 1999-02-10 Revised Date: 2007-11-15 |
Medline Journal Info:
|
Nlm Unique ID: 9010756 Medline TA: J Cardiovasc Electrophysiol Country: UNITED STATES |
Other Details:
|
Languages: eng Pagination: 1225-32 Citation Subset: IM |
Affiliation:
|
Department of Cardiovascular Diseases, King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Atrioventricular Node
/
physiopathology* Bundle of His / physiopathology Bundle-Branch Block / physiopathology Electrocardiography Family Follow-Up Studies Heart Conduction System / physiopathology* Humans Infant Long QT Syndrome / complications, genetics, physiopathology* Male Prognosis Purkinje Fibers / physiopathology Terminology as Topic |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Retrograde Purkinje potential activation during sinus rhythm following catheter ablation of idiopath...
Next Document: The architecture of the sinus node, the atrioventricular conduction axis, and the internodal atrial ...