| Pharmacological and non-pharmacological management of the congenital long QT syndrome: the rationale. | |
| | |
MedLine Citation:
|
PMID: 21396958 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
The congenital long QT syndrome (LQTS) is a familial disorder characterized by a prolongation of the QT interval on the ECG and occurrence of life-threatening cardiac arrhythmias especially, but not only, under conditions of increased sympathetic activity. Symptomatic untreated patients are at high risk for sudden cardiac death. Twelve LQTS genes have been identified and most of them encode cardiac ion channels. Very effective therapies are available and in carefully treated patients mortality is around 0.5-1% over 20 years. The initial treatment should always involve β-blockers, with propranolol and nadolol being the two most effective ones. With few exceptions all mutation carriers should be treated because of the risk of sudden death during the first cardiac event. Approximately 20% of patients continue to have syncope despite the β-blockers and the most rationale next level of therapy is represented by Left Cardiac Sympathetic Denervation (LCSD), which is highly effective and can complement any other therapy. One important limitation of LCSD is that, without valid reasons, it is available only in a few selected centers. Whenever syncope recurs despite LCSD, or whenever an aborted cardiac arrest has occurred, it becomes logical to resort to the implantation of a cardioverter defibrillator (ICD). The latter, however, is burdened by a high rate (31%) of adverse events including severe ones such as endocarditis, inappropriate shocks, and by the need of frequent battery replacements. A scoring system, based on simple clinical variables, can identify the patients more and less likely to benefit from ICD implantation. |
| | |
Authors:
|
Peter J Schwartz |
Related Documents
:
|
21143858 - Cardiogenic shock associated with loco-regional anesthesia rescued with left ventricula... 21336538 - Current state of ventricular assist devices. 2920408 - Changes in creatine kinase activity in the course of acute myocardial infarction. 12442928 - Myocardial ischaemia in patients with impaired left ventricular function undergoing cor... 20930148 - Angiotensin-converting enzyme inhibition prevents the release of monocytes from their s... 12503798 - Long-term results after ankle arthrodesis: clinical, radiological, gait analytical aspe... |
Publication Detail:
|
Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2011-03-17 |
Journal Detail:
|
Title: Pharmacology & therapeutics Volume: 131 ISSN: 1879-016X ISO Abbreviation: Pharmacol. Ther. Publication Date: 2011 Jul |
Date Detail:
|
Created Date: 2011-05-23 Completed Date: 2011-10-05 Revised Date: 2011-11-24 |
Medline Journal Info:
|
Nlm Unique ID: 7905840 Medline TA: Pharmacol Ther Country: England |
Other Details:
|
Languages: eng Pagination: 171-7 Citation Subset: IM |
Copyright Information:
|
Copyright © 2011 Elsevier Inc. All rights reserved. |
Affiliation:
|
Department of Lung, Blood and Heart, Section of Cardiology, University of Pavia, Pavia, Italy. peter.schwartz@unipv.it |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adrenergic beta-Antagonists
/
therapeutic use Death, Sudden, Cardiac / etiology Defibrillators, Implantable / adverse effects Humans Long QT Syndrome / congenital*, drug therapy, genetics, therapy* Nadolol / therapeutic use Propranolol / therapeutic use |
| Grant Support | |
ID/Acronym/Agency:
|
GGP07016//Telethon; GGP09247//Telethon; HL-68880/HL/NHLBI NIH HHS; R01 HL068880-01/HL/NHLBI NIH HHS; R01 HL068880-10/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
|
0/Adrenergic beta-Antagonists; 42200-33-9/Nadolol; 525-66-6/Propranolol |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Lack of association of tcdc type and binary toxin status with disease severity and outcome in toxige...
Next Document: Estimating the duration of intracellular action potentials in muscle fibres from single-fibre extrac...