Association of Sudden Death and Seizure with Left Ventricle Non-compaction in a Family.
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.


METHODS
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.


CONCLUSIONS
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.
Department of Pediatrics, Tehran University of Medical Sciences, Tehran, IR Iran ; Children's Medical Center, Pediatric Center of Excellence, Tehran, IR Iran.
Arman
A
Masihi Kocharian
Ahmad
A
Gamei Khosroshahi
Elahe
E
Malakan-Rad
Behzad
B
Mohammadpour Ahranjani