Document Detail

Association of Sudden Death and Seizure with Left Ventricle Non-compaction in a Family.
MedLine Citation:
PMID:  23056731     Owner:  NLM     Status:  PubMed-not-MEDLINE    
BACKGROUND: Left ventricle non-compaction (LVNC) or persistence of spongy myocardium is a rare form of congenital cardiomyopathy which presents with cardiac failure, thromboembolic events, arrhythmia and sudden death.
CASE PRESENTATION: We report a family with two deceased children and two alive offspring diagnosed with left ventricular (LV) non-compaction. A 9-year-old boy and a 16-year-old girl of our reported family suddenly died following exercise and emotional provocation. Both had a history of convulsion and syncope, with the latter taking anticonvulsant medication. Following their demise, the other members of the family were evaluated by echocardiography. The remaining two offsprings, one boy and one girl were diagnosed as having isolated LVNC.
CONCLUSION: Syncope and convulsion can be first manifestations of LVNC and heralding signs for sudden death in patients with LVNC. Echocardiography can be helpful for early diagnosis.
Arman Masihi Kocharian; Ahmad Gamei Khosroshahi; Elahe Malakan-Rad; Behzad Mohammadpour Ahranjani
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Iranian journal of pediatrics     Volume:  20     ISSN:  2008-2142     ISO Abbreviation:  Iran J Pediatr     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2012-10-11     Completed Date:  2012-10-12     Revised Date:  2013-05-30    
Medline Journal Info:
Nlm Unique ID:  101274724     Medline TA:  Iran J Pediatr     Country:  Iran    
Other Details:
Languages:  eng     Pagination:  363-6     Citation Subset:  -    
Department of Pediatrics, Tehran University of Medical Sciences, Tehran, IR Iran ; Children's Medical Center, Pediatric Center of Excellence, Tehran, IR Iran.
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