Document Detail


Isolated left ventricular noncompaction syndrome.
MedLine Citation:
PMID:  19801037     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by prominent trabeculae, deep intertrabecular recesses, and thickened myocardium with 2 distinct layers (compacted and noncompacted). Clinical characteristics, outcomes, and appropriate therapies remain poorly defined. Data were collected on patients diagnosed with ILVNC by echocardiographic criteria at the Mayo Clinic from 2001 through 2006. These data were entered prospectively into a clinical database and retrospectively analyzed. All-cause mortality, stroke, and development of atrial fibrillation (AF) were compared to community and nonischemic dilated cardiomyopathic (DC) controls. Implantable cardioverter-defibrillator (ICD) therapies were examined. Thirty patients with confirmed ILVNC were included in analyses (mean age at diagnosis 39 +/- 19.5 years, 60% men). Three patients with ILVNC died during follow-up (mean 2.5 +/- 1.2 years) compared to 5 DC and 1 community controls. No mortality difference was observed among these groups (p = 0.42 and 0.054, respectively). No ILVNC deaths were observed in patients with normal LV ejection fraction. New-onset AF was diagnosed in 2 patients with ILVNC, and none was observed in DC controls. Stroke occurred in 2 DC controls and none was observed in patients with ILVNC. ICDs were implanted in 11 patients with ILVNC. No appropriate therapies were identified during follow-up, but 2 patients underwent inappropriate therapies related to AF. In conclusion, mortality in patients with ILVNC is similar to that in DC patients. Deaths were observed only in patients with decreased LV ejection fraction, suggesting that ICD therapy may be reserved for this subgroup. New-onset AF may lead to inappropriate ICD discharges.
Authors:
Christopher Stanton; Charles Bruce; Heidi Connolly; Peter Brady; Imran Syed; David Hodge; Samuel Asirvatham; Paul Friedman
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Publication Detail:
Type:  Comparative Study; Journal Article     Date:  2009-08-28
Journal Detail:
Title:  The American journal of cardiology     Volume:  104     ISSN:  1879-1913     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-10-05     Completed Date:  2009-10-20     Revised Date:  2010-04-12    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1135-8     Citation Subset:  AIM; IM    
Affiliation:
Mayo Clinic, Rochester, Minnesota.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Cardiomyopathies / congenital*,  diagnosis,  mortality
Diagnosis, Differential
Echocardiography / methods
Electrocardiography
Female
Follow-Up Studies
Heart Ventricles / abnormalities*,  physiopathology
Humans
Male
Middle Aged
Minnesota / epidemiology
Prognosis
Retrospective Studies
Stroke Volume
Survival Rate / trends
Syndrome
Ventricular Dysfunction, Left / diagnosis,  etiology*,  mortality
Young Adult
Comments/Corrections
Comment In:
Am J Cardiol. 2010 Apr 15;105(8):1205-6   [PMID:  20381682 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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