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J Card Fail. 2011 Sep;17(9):771-8. doi: 10.1016/j.cardfail.2011.05.002.

Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview.

Author information

1
Cardiovascular Division, Mayo Clinic Arizona, Scottsdale, Arizona 85259-5499, USA.

Abstract

BACKGROUND:

Owing to inconsistent diagnostic criteria and small heterogeneous cohorts, little is known about the long-term outcomes of adult left ventricular noncompaction (LVNC), a rare cardiomyopathy with potentially serious outcomes. This systematic overview aimed to better delineate the natural history of adult LVNC.

METHOD AND RESULTS:

A comprehensive computerized search using "noncompaction" and its synonyms initially identified 206 articles, with reference lists subsequently hand scanned. These searches yielded 5 studies that were eligible for this systematic overview, identifying adult cohorts with isolated LVNC diagnosed by similar echocardiographic criteria. This combined cohort (n = 241) was followed for a mean duration of 39 months. The annualized event rate was 4% for cardiovascular deaths, 6.2% for cardiovascular death and its surrogates (heart transplantation and appropriate implantable cardioverter-defibrillator shocks), and 8.6% for all cardiovascular events (death, stroke, implantable cardioverter-defibrillator shocks, and heart transplantation.) Familial occurrence of LVNC in first-degree relatives was identified by echocardiography in 30% of index cases who were screened.

CONCLUSION:

LVNC is an increasingly recognized cardiomyopathy diagnosed by echocardiography and is associated with familial tendencies, arrhythmias, thromboembolism, advanced heart failure, and death.

PMID:
21872148
DOI:
10.1016/j.cardfail.2011.05.002
[Indexed for MEDLINE]

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