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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 2008-2150
Vol. 20, No. 3, 2010, pp. 363-366
Bioline Code: pe10055
Full paper language: English
Document type: Case Report
Document available free of charge

Iranian Journal of Pediatrics, Vol. 20, No. 3, 2010, pp. 363-366

 en Association of Sudden Death and Seizure with Left Ventricle Non-compaction in a Family
Kocharian, Arman Masihi; Khosroshahi, Ahamd Gamei; Malakan-Rad, Elahe & Ahranjani, Behzad Mohammadpour


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.

Cardiomyopathies; Non-compaction of the Ventricular Myocardium; Sudden death; Syncope

© Copyright 2010 Iran Journal of Pediatrics.
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