Isolated noncompaction of the ventricular myocardium.

Isolated noncompaction of the ventricular myocardium.

Dass B; Juszczyk MA; Hoffman D; Young JB

Hospital Physician

2008

2008-04

Noncompacted ventricular myocardium (NCVM) is a rare congenital cardiomyopathy. Anatomically, NCVM is characterized by an excessive number of deep trabeculations in the myocardial wall that communicate with the ventricular chamber. Although NCVM is typically diagnosed in pediatric patients, onset of symptoms of cardiomyopathy can be delayed into adulthood. Diagnosis is often established by typical spongiform appearance on 2-dimensional echocardiography and magnetic resonance imaging. Most presentations involve heart failure, arrhythmias, or nonspecific symptoms. In this article, the authors report the case of a man who was diagnosed with NCVM in late adulthood and was subsequently found to have a strong family history of heart disease.

Male; Middle Age; Defibrillators; Congenital; Heart Defects; Myocardial Diseases; Implantable; Congenital – Diagnosis; Adrenergic Beta-Antagonists – Therapeutic Use; Angiotensin-Converting Enzyme Inhibitors – Therapeutic Use; Congenital – Therapy; Diuretics – Therapeutic Use

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