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Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview
Nisha L Bhatia1, A Jamil Tajik, Susan Wilansky
1Cardiovascular Division, Mayo Clinic Arizona, Scottsdale, Arizona 85259-5499, USA.
Insights
Adult left ventricular noncompaction (LVNC) outcomes are better understood through this systematic overview. This rare cardiomyopathy, diagnosed by echocardiography, shows significant risks of cardiovascular events and familial tendencies.
Area of Science:
- Cardiology
- Genetics
- Echocardiography
Background:
- Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with poorly understood long-term outcomes due to inconsistent diagnostic criteria and small patient cohorts.
- This condition can lead to serious cardiac events.
- Understanding the natural history of adult LVNC is crucial for patient management.
Purpose of the Study:
- To systematically review and delineate the long-term outcomes and natural history of adult left ventricular noncompaction (LVNC).
- To consolidate data from eligible studies to provide a clearer picture of LVNC prognosis.
Main Methods:
- A systematic overview was conducted using comprehensive computerized searches for "noncompaction" and related terms.
- Reference lists of identified articles were hand-scanned to find additional eligible studies.
- Data from 5 studies, comprising a combined cohort of 241 adult patients with isolated LVNC diagnosed via similar echocardiographic criteria, were analyzed.
Main Results:
- The combined cohort (n=241) was followed for a mean of 39 months.
- Annualized event rates included 4% for cardiovascular deaths, 6.2% for major adverse cardiovascular events (death, transplantation, ICD shocks), and 8.6% for all cardiovascular events (death, stroke, transplantation, ICD shocks).
- Echocardiographic screening revealed familial occurrence of LVNC in 30% of index cases.
Conclusions:
- Left ventricular noncompaction (LVNC) is an increasingly recognized cardiomyopathy diagnosed via echocardiography.
- LVNC is associated with familial tendencies, arrhythmias, thromboembolism, advanced heart failure, and increased mortality.
- The findings highlight the significant cardiovascular risks and hereditary nature of LVNC.
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.
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