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Published on: April 8, 2013
Left ventricular noncompaction: a 25-year odyssey
Timothy E Paterick1, Matt M Umland, M Fuad Jan
1Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway #845, Milwaukee, WI 53215, USA. publishing15@aurora.org
Insights
Left ventricular noncompaction (LVNC) is a heart muscle disease with varied symptoms and risks. Advances in imaging like echocardiography and cardiac MRI enhance diagnosis and understanding of this condition.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Left ventricular noncompaction (LVNC) is a primary cardiomyopathy.
- It can be sporadic or familial, often with autosomal dominant inheritance.
- Clinical presentations range from asymptomatic to severe heart failure, arrhythmias, and sudden cardiac death.
Purpose of the Study:
- To summarize the current understanding of LVNC.
- To highlight diagnostic modalities and their advancements.
- To underscore the progress in comprehending this heterogeneous cardiac condition.
Main Methods:
- Echocardiography is the primary diagnostic tool.
- Cardiac magnetic resonance imaging (CMR) aids in staging disease severity.
- Research integrates embryology, imaging, and genetics.
Main Results:
- Echocardiography reveals a thick, bilayered myocardium with prominent trabeculations and deep recesses.
- CMR improves disease staging and prediction of adverse events.
- Increased awareness and improved imaging technologies are enhancing LVNC diagnosis.
Conclusions:
- LVNC is a complex cardiomyopathy with significant clinical implications.
- Advancements in echocardiography and CMR have revolutionized its diagnosis and management.
- Ongoing research in genetics and embryology continues to deepen our understanding of LVNC's heterogeneity.
Abstract:
Left ventricular noncompaction (LVNC) is a cardiomyopathy associated with sporadic or familial disease, the latter having an autosomal dominant mode of transmission. The clinical features associated with LVNC vary from asymptomatic to symptomatic patients, with the potential for heart failure, supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. Echocardiography is the diagnostic modality of choice, revealing the pathognomonic features of a thick, bilayered myocardium; prominent ventricular trabeculations; and deep intertrabecular recesses. Widespread use and advances in the technology of echocardiography and cardiac magnetic resonance imaging are increasing awareness of LVNC, and cardiac magnetic resonance imaging is improving the ability to stage the severity of the disease and potential for adverse clinical consequences. Study of LVNC through research in embryology, imaging, and genetics has allowed enormous strides in the understanding of this heterogeneous disease over the past 25 years.
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