Isolated left ventricular noncompaction: a review of three different presentations for a rare disease

Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy with a genetic background characterized by numerous prominent trabeculations and deep intertrabecular recesses. It occurs in the absence of any coexisting congenital lesion or hemodynamic abnormality and is rare in the adult po...

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Bibliographic Details
Published inConnecticut medicine Vol. 77; no. 1; p. 11
Main Authors Amuchástegui, Marcos, Amuchastegui, Tomas, Contreras, Alejandro Ernesto, Donohue, Thomas, Ghantous, Andre
Format Journal Article
LanguageEnglish
Published United States 01.01.2013
Subjects
Adolescent
Adult
Defibrillators, Implantable
Echocardiography, Doppler
Humans
Isolated Noncompaction of the Ventricular Myocardium - diagnosis
Isolated Noncompaction of the Ventricular Myocardium - diagnostic imaging
Isolated Noncompaction of the Ventricular Myocardium - pathology
Isolated Noncompaction of the Ventricular Myocardium - therapy
Male
Middle Aged
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Summary:Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy with a genetic background characterized by numerous prominent trabeculations and deep intertrabecular recesses. It occurs in the absence of any coexisting congenital lesion or hemodynamic abnormality and is rare in the adult population. Heart failure, ventricular arrhythmias, and embolic events are the three major clinical manifestations of ILVNC. Medical and surgical treatment is similar to other systolic dysfunction cardiomyopathies and depends on the presenting clinical manifestations. In this review, we present three patients with different clinical presentations of ILVNC leading to different treatment modalities ranging from medications alone to device implantation and transplantation. Pathologic findings from one of our patients are also presented.
ISSN:0010-6178