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Left ventricular non compaction in children
Sara H Weisz1, Giuseppe Limongelli, Giuseppe Pacileo
1Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
Insights
Left ventricular non compaction (LVNC) is a heart muscle condition with deep recesses in the left ventricle, increasing clot risk. This review focuses on sparse data regarding childhood LVNC cases.
Area of Science:
- Cardiology
- Developmental Biology
- Genetics
Background:
- Left ventricular non compaction (LVNC) is a myocardial disease defined by a hypertrabeculated left ventricular wall.
- Deep recesses in the myocardium increase the risk of blood clot formation.
- While adult LVNC is studied, data on childhood LVNC is limited.
Purpose of the Study:
- To review and summarize current clinical and preclinical knowledge of LVNC specifically in children.
- To highlight the sparse data available on pediatric LVNC.
- To consolidate information for better understanding and management of childhood LVNC.
Main Methods:
- Literature review of clinical and preclinical studies on LVNC.
- Focus on studies reporting on pediatric populations.
- Synthesis of findings related to diagnosis, presentation, and outcomes in children.
Main Results:
- LVNC presents with a hypertrabeculated myocardium, particularly affecting the left ventricular apex and free wall.
- The condition is likely due to premature interruption of normal heart development, specifically myocardial compaction.
- Existing research on pediatric LVNC is sparse, necessitating further investigation.
Conclusions:
- Understanding the developmental origins of LVNC is crucial.
- Further research is essential to address the paucity of data on childhood LVNC.
- Consolidating existing knowledge is a key step towards improving diagnosis and management in pediatric patients.
Abstract:
Left ventricular non compaction (LVNC) is a myocardial disease characterized by a hypertrabeculated myocardium. The hypertrabeculations in the left ventricular wall define deep recesses communicating with the left ventricular chamber where blood penetrates with increased risk of blood clots in the meshwork of the prominent trabeculations. The left ventricular apex and the free wall are particularly affected. During in utero ventriculogenesis, myocardial blood supply is initially linked to the presence of sinusoids, in which blood penetrates and diffuses nutriments and oxygen to myocardial cells. Progressively, with the development of the heart and the increase of cells demand of blood, coronary arteries system develops. This step is associated with myocardial modification that leads to compaction of hypertrabeculated myocardial net. Probably, the premature interruption of this process leads to ventricular noncompaction. Many studies have been conducted in adults with hypertrabeculated myocardium. To date, data regarding childhood LVNC are sparse. The aim of this review is to summarize the clinical and preclinical knowledge about LVNC in children.
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