ABSTRACT
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. We report a family with two deceased children and two alive offspring diagnosed with 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. 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