Isolated left ventricular non-compaction (LVNC) is a rare genetic form of cardiomyopathy (CM) characterized by prominent left ventricular wall trabeculation and intertrabecular recesses communicating with the ventricular cavity. Clinical signs are variable, ranging from lack of symptoms to severe manifestations including heart failure, sustained ventricular arrhythmias, cardioembolism and sudden death. The diagnosis of LVNC is frequently missed, due to limited awareness in the medical community. Contemporary diagnostic sensitivity has been enhanced by the introduction of specific morphologic criteria by high resolution echocardiography and cardiac magnetic resonance. As a consequence, LVNC has been diagnosed more frequently in association with other disorders such as congenital heart disease or genetic CM. This concept may prove relevant to the understanding of common pathogenetic mechanisms of CM and offer novel research opportunities. © 2010 AIM Publishing Srl.
Clinical and genetic features of left ventricular noncompaction: A continuum in cardiomyopathies / Baldi M., Sgalambro A., Nistri S., Girolami F., Baldini K., Fantini S., Grifoni C., Rega L., Olivotto I., Cecchi F.. - In: GIORNALE ITALIANO DI CARDIOLOGIA. - ISSN 1827-6806. - ELETTRONICO. - 11:(2010), pp. 377-385.
Clinical and genetic features of left ventricular noncompaction: A continuum in cardiomyopathies
Sgalambro A.;Rega L.;Olivotto I.;Cecchi F.
2010
Abstract
Isolated left ventricular non-compaction (LVNC) is a rare genetic form of cardiomyopathy (CM) characterized by prominent left ventricular wall trabeculation and intertrabecular recesses communicating with the ventricular cavity. Clinical signs are variable, ranging from lack of symptoms to severe manifestations including heart failure, sustained ventricular arrhythmias, cardioembolism and sudden death. The diagnosis of LVNC is frequently missed, due to limited awareness in the medical community. Contemporary diagnostic sensitivity has been enhanced by the introduction of specific morphologic criteria by high resolution echocardiography and cardiac magnetic resonance. As a consequence, LVNC has been diagnosed more frequently in association with other disorders such as congenital heart disease or genetic CM. This concept may prove relevant to the understanding of common pathogenetic mechanisms of CM and offer novel research opportunities. © 2010 AIM Publishing Srl.
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