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 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 .
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 .
isolated left ventricular non - compaction ( lvnc ) is one of the rare genetic heart diseases recently known , and characterized by heterogeneous defects in endocardium accompanied by increased trabeculation due to abnormal embryogenesis of the normal myocardium . in this disease ,
the myocardium is composed of two layers : spongy or non - compacted and normal or compacted ones .
the clinical manifestations include symptoms and signs of left ventricular failure ( common ) , those of right ventricle ( rare ) , ventricular arrhythmias , sudden death , mural thrombi and embolic events with cardiac origin .
we report a family having the history of two offspring deaths , at the ages of 9 and 16 , with two live ones suffering from lvnc .
we reported sudden death of a 9-year old boy with a history of two episodes of unprovoked convulsion and syncope in school and his 16-year old sister due to sudden death while she was getting back home after a feast . a 12year - old boy and a 7-year - old girl
were referred to pediatric cardiology clinic for cardiac evaluation following occurrence of sudden death in their siblings .
the first pregnancy of the mother ended in abortion at the age of two months of gestation , and the second offspring was a girl who died one week after birth following an operation to repair esophageal atresia .
the first victim of suspected lvnc in this family was a nine year old boy with a history of two episodes of unprovoked convulsion and syncope in school .
the first attack was in break time with spasm of neck and body , ended uneventfully .
the next one was during exercise , with sudden fall but no urinary incontinence , eye deviation or sialorrhea . the electrocardiogram ( ecg ) , electroencephalography and lab tests showed no abnormalities at that time .
the family lost their 16-year old daughter due to sudden death while she was getting back home after a feast .
she also had experienced two seizure episodes associated with loss of consciousness , one at the age of 9 during exercise at school and the other at the age of 10 on vacation .
both episodes had ended uneventfully , and brain ct scan with contrast demonstrated no pathologic finding .
the 12-year - old boy had no history of seizure , syncope , easy fatigability , exertional dyspnea or hospitalization .
on 2d transthorasic echocardiography , the parasternal short axis and four chamber views demonstrated increased left ventricular ( lv ) wall thickness accompanied by trabeculations and deep recesses that were indicative of lv non - compaction .
four chamber and short axis views demonstrating the ratio of non - compacted to normally compacted segments the coronary arteries were normal and no pericardial effusion and intracavitary thrombi were detected
. the 7-year - old girl also had no positive history of hospitalization , seizure , easy fatigue .
her ecg was normal , however , her 2d echocardiograms were compatible with the diagnosis of lv non - compcation , based on the diagnostic criteria already reported by frischknecht et al , with increased lateral , inferior and apical wall thickness and deep recesses and trabeculations .
the coronary arteries were normal and no pericardial effusion and intracavitary thrombi were detected ( fig .
parasternal long axis view and m - mode echocardiography showing abnormal trabeculation and spongy pattern of left ventricle the parents were evaluated clinically and by echocardiography .
this case report highlights the importance of syncope and seizure as heralding signs for sudden death in two deceased children of a family with two offspring having documented lv non - compaction .
although syncope and sudden death have been previously reported in adults with lvnc but to date , as to best of our knowledge , sudden death with prior heralding sign of syncope and seizure has not been reported in children with lv non - compaction . in our reported family
, both of the unexpected deaths occurred after previous episodes of uneventful seizure and syncope . considering the common embryologic origins of central nervous system and myocardium , occurrence of syncope and seizure in lvnc could be explained .
lv non - compaction or persistence of spongy myocardium , first described in 1984 , is a rare form congenital cardiomyopathy with a prevalence of 0.05 - 0.24/year .
the limitation of our report is lack of echocardiographic evidence for confirmation of diagnosis in the two deceased and not timely - referred and timely - diagnosed children .
we suggest careful biannual follow up ( ecg , holter monitoring , echocardiography ) and anticoagulation therapy ( aspirin , dose : 3 - 5mg/ kg / daily ) for alive sibling .
unexplained seizure and syncope in a child , particularly in a familial pattern , may warrant further echocardiographic evaluation for early diagnosis of left ventricular non - compaction to prevent catastrophic events .
although the numbers of our patients are limited , but it seems that seizure and syncope can be considered as warning signs in those patients with lvnc who are at high risk for sudden death .
 backgroundleft 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 presentationwe 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.conclusionsyncope 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 . 
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