Association of Sudden Death and Seizure with Left Ventricle Non-compaction in a Family
نویسندگان
چکیده
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.
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Non-compaction of the left ventricle (NCLV) was categorised as unclassified cardiomyopathy by the World Health Organization in 1995. Over the last decade this condition has been identified as a distinct form of cardiomyopathy and a genetically heterogeneous disorder. Clinically, this may be coupled with the loss of contractility, arrhythmia, and thromboembolism. The prognosis in a symptomatic p...
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Left ventricular non-compaction (LVNC) is a genetic cardiomyopathy often familial and autosomal dominant. It is characterized by morphological abnormalities affecting the left ventricular myocardium with prominent trabeculations of the inner surface of the ventricle, often extending deep into the ventricular wall. There are no pathognomonic histological findings with normal myocytes being inter...
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