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Start free with EleplanTriglyceride deposit cardiomyovasculopathy
ORPHA:692305Disease
Also called Neutral lipid storage disease with severe cardiovascular involvement · TGCV
What it is
A rare cardiovascular disorder characterized by defective intracellular lipolysis of long-chain triglycerides (LCTG), resulting in heart failure and diffuse atherosclerosis.
Key facts
- Inheritance
- Autosomal recessive, Unknown
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- <1 / 1 000 000Neutral lipid storage disease
- Age of onset
- Infancy, NeonatalNeutral lipid storage disease
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Genes reported in subtypes
Orphanet records this gene on 1 more specific entry under this disorder, not on this entry itself:
A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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