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Left Ventricular Hypertrabeculation: Historical Evolution, Diagnostic Pitfalls, and a Pragmatic Clinical Approach
Alessandro Parodi1, Marika Martini1, Annagrazia Cecere1
1Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua Medical School, 35128 Padua, Italy.
Insights
Left ventricular hypertrabeculation (LVHT) is a myocardial feature now viewed as a trait, not a distinct cardiomyopathy. Further research is needed to clarify its associations, mechanisms, and improve diagnostic accuracy.
Area of Science:
- Cardiology
- Genetics
- Molecular Biology
Background:
- Left ventricular hypertrabeculation (LVHT), formerly left ventricular non-compaction (LVNC), has evolving diagnostic criteria.
- Current guidelines classify LVHT as a myocardial trait rather than a primary genetic cardiomyopathy.
- Incomplete understanding persists regarding LVHT's pathophysiology, genetic basis, and associations with other syndromes.
Purpose of the Study:
- To review the diagnostic criteria, pathophysiology, and management strategies for LVHT.
- To address the diagnostic uncertainties and clinical challenges associated with LVHT.
- To propose a practical clinical approach for managing patients with LVHT.
Main Methods:
- Systematic review of existing literature on LVHT.
- Analysis of diagnostic criteria and classification changes over time.
- Examination of pathophysiological mechanisms and genetic underpinnings.
- Evaluation of current management guidelines and clinical data.
Main Results:
- LVHT recognition and classification have fluctuated, shifting from a cardiomyopathy to a trait.
- Data linking LVHT to other syndromes and its molecular mechanisms remain inconclusive.
- Accurate diagnosis is crucial to avoid over- or underdiagnosis and manage uncertainties.
Conclusions:
- LVHT is a complex myocardial feature requiring further investigation.
- Standardized data collection is essential for improved diagnostic accuracy and management.
- A practical approach is proposed to aid clinicians in diagnosing and managing LVHT.
Abstract:
Left ventricular hypertrabeculation (LVHT), previously referred to as left ventricular non-compaction (LVNC), has experienced fluctuating recognition in cardiology. Once defined as a primary genetic cardiomyopathy, this intriguing myocardial feature is now considered a "trait" in the most recent guidelines. However, the understanding of this phenomenon remains incomplete. Moreover, data on the association between this feature and other syndromes are inconclusive, and further advances in understanding the associated molecular mechanisms and genetic background are needed. A systematic collection of clinical data is essential to avoid both over- and underdiagnosis, thereby reducing current uncertainties in LVHT management. This review examines the diagnostic, pathophysiological, and management aspects of LVHT, highlighting the challenging nature of this feature and proposing a practical approach for clinicians.
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