Aurora 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.
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.
"Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology, which is most frequently diagnosed by echocardiography, but can be also visualized by cardiac computed tomography, magnetic resonance imaging or ventriculography . If systematically screened, LVHT is frequently associated with neuromuscular disorders (NMD) . "
[Show abstract][Hide abstract] ABSTRACT: Aim of this study was to assess pathomorphologic findings (PATHO) in patients with echocardiographically (ECHO) diagnosed left ventricular hypertrabeculation/noncompaction.
ECHO-criteria for LVHT were: >3 trabeculations, moving synchronously with the compacted myocardium, and forming the noncompacted part of a two-layered myocardium. At autopsy, the hearts were investigated according to the pathologists' preferences.
Twelve patients (2 females, age 27-81years) were included. Seven suffered from neuromuscular disorders, 5 patients were not investigated neurologically. The specimens were acquired after explantation during heart transplantation (n=1), death due to heart failure (n=6), sudden death (n=2), pneumonia (n=2) and stroke (n=1). Eight hearts were investigated without fixation and 4 after formaldehyde fixation. The hearts were opened along the long-axis, in 3 hearts additional short-axis cuts were carried out. At PATHO the trabecular meshwork was better visible in the formaldehyde-fixed hearts than in the fresh hearts. Differentiation from papillary muscles was easier on the long-axis cuts, whereas the two-layered structure was better visible on short-axis cuts. The trabecular pattern was similar in patients with neuromuscular disorders and those who did not undergo neurologic investigation. Subendocardial fibrosis was found in each case. Due to the complex three-dimensional geometry, it was impossible to count the number of trabeculations.
Formaldehyde-fixation should be performed when comparing ECHO with PATHO findings in LVHT. Long-axis as well as short-axis cuts should be carried out in order to assess the course of trabeculations and the extent of the two-layered structure. Subendocardial fibrosis in LVHT deserves further research.
International journal of cardiology 09/2013; 169(4). DOI:10.1016/j.ijcard.2013.08.138 · 4.04 Impact Factor
"At 5 weeks of development in humans, trabeculae are present, and at 5–8 weeks of development, the process of trabecular remodeling and compaction occurs, as the coronary arterial circulation develops . Compaction starts at the base of the heart and continues towards the apex, which may explain why noncompaction most prominently involves the midventricle to apex of the heart [Paterick et al., 2012]. "
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