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Review
. 2010 Mar;107(12):206-13.
doi: 10.3238/arztebl.2010.0206. Epub 2010 Mar 26.

Isolated non-compaction cardiomyopathy

Rolf Engberding  1 , Claudia Stöllberger, Peter Ong, Talat M Yelbuz, Birgit J Gerecke, Günter Breithardt
Affiliations
Review

Isolated non-compaction cardiomyopathy

Rolf Engberding et al. Dtsch Arztebl Int. 2010 Mar.

Abstract

Background: Isolated non-compaction cardiomyopathy (NCCM) was first described in 1984. This disorder, a primary genetic cardiomyopathy, is now attracting increased attention.

Method: The current state of the epidemiology, pathogenesis, pathophysiology, clinical features, diagnosis, treatment, and prognosis of NCCM are discussed on the basis of a review of selected literature as well as the authors' personal experience.

Results: The pathogenesis of NCCM is thought to involve a genetically determined disturbance of the myocardial compaction process during fetal endomyocardial morphogenesis. It is not accompanied by any other cardiac anomalies. Echocardiography is the diagnostic method of choice. The diagnosis is based on the following echocardiographic criteria: the presence of at least 4 prominent trabeculations and deep intertrabecular recesses, blood flow from the ventricular cavity into the intertrabecular recesses, and a typical bilaminar structure of the affected portion of the left ventricular myocardium. NCCM can also be diagnosed with magnetic resonance imaging of the heart. The clinical severity of NCCM is variable; its manifestations include heart failure, thromboembolic events, and arrhythmias. The treatment is symptom-based. Patients with symptomatic NCCM have a poor prognosis.

Conclusion: NCCM is a type of cardiomyopathy that was first described 25 years ago. Its molecular genetic basis is not yet fully clear, and the same is true of its diagnosis, treatment, and prognosis. Further study of these matters is needed.

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Figures

Figure 1
Figure 1
Dissected autopsy specimen: the heart of a patient with isolated non-compaction cardiomyopathy. There is extensive trabecula formation in the left ventricular wall. The arrow points to a thrombus in the trabecular meshwork
Figure 2
Figure 2
TEE demonstration of the short axis of the left ventricle, visualized by transgastric insonation. Color Doppler flow ultrasonography reveals blood flow between the extensive trabecula in this patient with isolated NCCM
Figure 3
Figure 3
Transthoracic echocardiographic view of the long axis of the heart in a patient with isolated NCCM, revealing the compact and non-compact layers of the left ventricle. c, compact layer; nc, non-compact layer
Figure 4
Figure 4
Magnetic resonance imaging of the heart (cardiac MRI) in a man with isolated non-compaction cardiomyopathy. Cine-SSFP images taken along the long and short axes are shown (left and right, respectively); arrow: increased trabeculation
Figure 5
Figure 5
Left ventriculogram of a woman with isolated non-compaction cardiomyopathy, showing excessive trabeculation.
Figure 6
Figure 6
Classification of primary cardiomyopathies by the American Heart Association (e48); HCM, hypertrophic cardiomyopathy; DCM, dilated cardiomyopathy; ARVC, arrhythmogenic right-ventricular cardiomyopathy; NCCM, isolated non-compaction cardiomyopathy
See this image and copyright information in PMC

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MeSH terms