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Updated: Mar 22, 2026
Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
[Left ventricular noncompaction: A clinical and morphological study]
L B Mitrofanova1, O M Moiseeva1, D A Khashchevskaya1
1V.A. Almazov Federal Medical Research Center, Saint Petersburg, Russia.
Insights
Left ventricular noncompaction (LVNC) involves significant noncompacted myocardium and often co-occurs with other cardiomyopathies. Findings suggest hemodynamic and inflammatory factors may influence LVNC development.
Area of Science:
- Cardiovascular Pathology
- Histopathology
- Molecular Cardiology
Context:
- Left ventricular noncompaction (LVNC) is a rare congenital heart defect characterized by excessive trabeculation.
- Understanding the diverse morphological spectrum and underlying mechanisms of LVNC is crucial for diagnosis and management.
Purpose:
- To comprehensively analyze the clinicopathological features of left ventricular noncompaction (LVNC).
- To investigate the association of LVNC with other cardiac pathologies and potential genetic factors.
- To correlate magnetic resonance imaging (MRI) findings with morphological data in LVNC patients.
Summary:
- Analysis of 7 LVNC patient hearts revealed a predominant noncompact layer, myocardial hypertrophy, fibrosis, and endocardial fibroelastosis in most cases.
- Associated findings included right ventricular hypertrabeculation, myocarditis, lipomatosis, and altered connexin 43 expression.
- Morphological findings aligned with MRI in 100% of cases, showing overlapping features with restrictive, hypertrophic, and dilated cardiomyopathies.
Impact:
- This study elucidates the complex histopathological landscape of LVNC, highlighting its frequent coexistence with other cardiomyopathies.
- The findings suggest that hemodynamic and inflammatory factors may play a significant role in the pathogenesis of the LVNC phenotype.
- Provides valuable insights for differential diagnosis and understanding the multifactorial etiology of LVNC.
Abstract:
The data of clinical, macro- and micrometric, histological, and immunohistochemical studies of the heart were analyzed in patients with left ventricular noncompaction (LVNC). Materials from 7 patients: 5 hearts of recipients after heart transplantation, one heart of a dead patient, and one endomyocardial biopsy specimen were investigated. The investigations showed that this disease was accompanied by a preponderance of a noncompact layer with its ratio to a compact layer (2.4:6.6) in the left ventricle and by myocardial hypertrophy and fibrosis in all cases, by endocardial fibroelastosis and discomplexation of muscle fibers by more than 15% of the specimen area in 6 of the 7 cases, by right ventricular hypertrabeculation and myocarditis in 5 cases, and by lipomatosis and impaired connexin 43 expression in 4 cases. Only one of the four patients was found to have MYH 7 gene mutation. The results of MRI of the extracted heart coincided with morphological findings in 100% of cases. The comparative study demonstrated that this disease had simultaneously morphological features of both LVNC and restrictive, hypertrophic, dilated cardiomyopathy. The findings may suggest that the LVNC phenotype may be formed under the influence of various modifying factors (hemodynamic and inflammatory ones).
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