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Isolated Noncompaction of the Ventricular Myocardium

Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. Often, the RIGHT VENTRICLE is also affected. CONGESTIVE HEART FAILURE; PULMONARY EMBOLISM; and ventricular ARRHYTHMIA are commonly seen.
Also Known As:
Isolated Non-compaction of the Ventricular Myocardium; Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked; Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant
Networked: 7 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Related Diseases

1. Ventricular Tachycardia
2. Cardiac Sudden Death (Sudden Cardiac Arrest)
3. Ventricular Fibrillation
4. Muscular Diseases (Myopathy)
5. Long QT Syndrome

Experts

1. Saito, Satoshi: 3 articles (08/2009 - 06/2006)
2. Matsumoto, Naoya: 3 articles (08/2009 - 06/2006)
3. Sato, Yuichi: 3 articles (08/2009 - 06/2006)
4. Kunimasa, Taeko: 2 articles (08/2009 - 05/2007)
5. Imai, Shinobu: 2 articles (05/2007 - 06/2006)
6. Onay, Ozge Surmeli: 1 article (01/2013)
7. Erdem, Sevcan: 1 article (01/2013)
8. Yildirim, Isil: 1 article (01/2013)
9. Karagoz, Tevfik: 1 article (01/2013)
10. Yilmaz, Mustafa: 1 article (01/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Isolated Noncompaction of the Ventricular Myocardium:
1. Brain Natriuretic Peptide (Natrecor)FDA Link
2. GadoliniumIBA

Therapies and Procedures

1. Defibrillators (Defibrillator)
2. Cardiac Resynchronization Therapy
3. Implantable Defibrillators (Implantable Cardioverter-Defibrillator)