| Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors With Preserved Ejection Fraction Registry (CASPER). | |
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MedLine Citation:
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PMID: 19597050 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Cardiac arrest without evident cardiac disease may be caused by subclinical genetic conditions. Provocative testing to unmask a phenotype is often necessary to detect primary electrical disease, direct genetic testing, and perform family screening. METHODS AND RESULTS: Patients with apparently unexplained cardiac arrest and no evident cardiac disease (normal cardiac function on echocardiogram, no evidence of coronary artery disease, and a normal ECG) underwent systematic evaluation that included cardiac magnetic resonance imaging, signal-averaged ECG, exercise testing, drug challenge, and selective electrophysiological testing. Diagnostic criteria were based on accepted criteria or provocation of the characteristic clinical features for long-QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, early repolarization, arrhythmogenic right ventricular cardiomyopathy, coronary spasm, and myocarditis. Sixty-three patients in 9 centers were enrolled (age 43.0+/-13.4 years, 29 women). A diagnosis was obtained in 35 patients (56%): Long-QT syndrome in 8, catecholaminergic polymorphic ventricular tachycardia in 8, arrhythmogenic right ventricular cardiomyopathy in 6, early repolarization in 5, coronary spasm in 4, Brugada syndrome in 3, and myocarditis in 1. Targeted genetic testing demonstrated evidence of causative mutations in 9 (47%) of 19 patients. Screening of 64 family members of these patients identified 15 affected individuals who were treated (24%). The remaining 28 patients (44%) were considered to have idiopathic ventricular fibrillation. CONCLUSIONS: Systematic clinical testing, including drug provocation and advanced imaging, results in unmasking of the cause of apparently unexplained cardiac arrest in >50% of patients. This approach assists in directing genetic testing to diagnose genetically mediated arrhythmia syndromes, which results in successful family screening. |
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Authors:
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Andrew D Krahn; Jeffrey S Healey; Vijay Chauhan; David H Birnie; Christopher S Simpson; Jean Champagne; Martin Gardner; Shubhayan Sanatani; Derek V Exner; George J Klein; Raymond Yee; Allan C Skanes; Lorne J Gula; Michael H Gollob |
Publication Detail:
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Type: Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't Date: 2009-07-13 |
Journal Detail:
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Title: Circulation Volume: 120 ISSN: 1524-4539 ISO Abbreviation: Circulation Publication Date: 2009 Jul |
Date Detail:
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Created Date: 2009-07-28 Completed Date: 2009-08-13 Revised Date: 2010-06-30 |
Medline Journal Info:
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Nlm Unique ID: 0147763 Medline TA: Circulation Country: United States |
Other Details:
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Languages: eng Pagination: 278-85 Citation Subset: AIM; IM |
Affiliation:
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University of Western Ontario, London, Ontario, Canada. akrahn@uwo.ca |
| Data Bank Information | |
Bank Name/Acc. No.:
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ClinicalTrials.gov/NCT00292032 |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Coronary Angiography / methods Female Heart Arrest / classification*, etiology*, physiopathology, radiography Humans Male Middle Aged Prospective Studies Registries* Stroke Volume* / physiology Survivors* Young Adult |
| Comments/Corrections | |
Erratum In:
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Circulation. 2010 Jun 29;121(25):e460 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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