Noncompaction of the Ventricular Myocardium,☆☆

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Abstract

Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular recesses communicating with the left ventricular cavity. The disease uniformly affects the left ventricle, with or without concomitant right ventricular involvement, and results in systolic and diastolic ventricular dysfunction and clinical heart failure. Noncompaction was initially described in children. However, recent studies have characterized this disease in the adult population, in whom this process may be more prevalent than currently appreciated. We describe an illustrative case of isolated noncompaction of the ventricular myocardium in a 57-year-old woman with the typical clinical and echocardiographic features of the disease. The literature on the topic is reviewed. (J Am Soc Echocardiogr 1999;12:859-63.)

Section snippets

History

A 57-year-old white woman was evaluated at our institution for progressive heart failure. She had no symptoms until age 51 years, when she began to have progressive left heart failure, intermittently associated with paroxysms of atrial fibrillation. The patient did not have chest pain, ventricular arrhythmias (symptomatic or detected with 24-hour Holter monitoring), or systemic embolic episodes. A dual-chamber pacemaker was implanted shortly after the onset of heart failure because of

Normal Versus Abnormal Endomyocardial Embryogenesis

During the first month of fetal life, before the development of the coronary circulation, the human embryonic myocardium consists of a “spongy” meshwork of interwoven myocardial fibers forming trabeculae with deep intertrabecular recesses.3, 4 These recesses communicate with the LV cavity, and blood is supplied to the myocardium through the intertrabecular spaces, resembling the myocardial blood supply in cold-blooded species. During weeks 5 to 8 of fetal life, 2 parallel processes normally

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Cited by (172)

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    Citation Excerpt :

    Although removing noncompacted trabeculae that cause thrombus formation can prevent embolism, the procedure may be used to treat heart failure by improving both the LV diastolic and systolic function.6-8 According to Agmon and colleagues,7 diastolic dysfunction in LVNC is related to both abnormal relaxation and restrictive filling caused by the numerous prominent and fibrous trabeculae. Therefore removing noncompacted trabeculae that restrict ventricular relaxation and filling might restore diastolic function.

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Reprint requests: James B. Seward, MD, Mayo Clinic, 200 First St SW, Rochester, MN 55905.

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