Isolated Noncompaction of the Left Ventricle in Adults.

Hussein, Ayman

Karimianpour, Ahmadreza

Collier, Patrick

Krasuski, Richard A

United States

2015-12-03T15:32:37Z

2015-08-04

Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy that was first described in 1926 as a "spongy myocardium." The disorder results from intrauterine arrest of compaction of the loose interwoven meshwork of the fetal myocardial primordium and subsequent persistence of deep trabecular recesses in the myocardial wall. The classical clinical presentation is a triad of heart failure, arrhythmias, and embolic events from mural thrombi. ILVNC has been associated with several autosomal dominant, X-linked, and mitochondrial genetic mutations that are also shared among other cardiomyopathies. Over the past decade, ILVNC has been subject to intensive research, as it increases the risk for sudden cardiac death. This review focuses on the current understanding of ILVNC in adult populations and attempts to provide organized insight into the disease process, screening, diagnosis, management, role of device therapy, and prognosis.

http://www.ncbi.nlm.nih.gov/pubmed/26227197

S0735-1097(15)02780-1

1558-3597

https://hdl.handle.net/10161/10986

eng

Elsevier BV

J Am Coll Cardiol

10.1016/j.jacc.2015.06.017

LVNC

congenital

heart failure

primordial

Adult

Death, Sudden, Cardiac

Defibrillators, Implantable

Disease Management

Disease Progression

Humans

Isolated Noncompaction of the Ventricular Myocardium

Prognosis

Isolated Noncompaction of the Left Ventricle in Adults.

Journal article

Krasuski, Richard A|0000-0003-3150-5215

http://www.ncbi.nlm.nih.gov/pubmed/26227197

578

585

5

Clinical Science Departments

Duke

Medicine

Medicine, Cardiology

School of Medicine

Published

66