ARVC (Arrhythmogenic Right Ventricular Cardiomyopathy)
Definition
Cardiomyopathy in which right ventricular myocardium is progressively replaced by fibrofatty tissue, predisposing to right ventricular arrhythmia and sudden death. Leading cause of SCD in young athletes in Italy (Corrado series). Easily missed at autopsy unless the right ventricle is specifically examined.
Related terms
- Channelopathy
- Ion-channel gene mutation causing potentially fatal arrhythmia without structural cardiac abnormality. The three principal forensic channelopathies are long QT syndrome (LQTS), Brugada...
- Hypertrophic Cardiomyopathy (HCM)
- Autosomal dominant structural cardiomyopathy characterised by asymmetric septal hypertrophy (septal:free-wall thickness ratio > 1.3) and microscopic myocyte disarray covering > 5 percent...
- Post-Mortem Genetic Testing (Cardiac)
- Gene-panel analysis of post-mortem tissue (frozen fresh myocardium or paraffin-embedded blocks) targeting ion-channel and cardiomyopathy genes. Recommended in the UK (RCPath inherited...
- Serum Tryptase
- Enzyme released from mast cells during degranulation. Post-mortem tryptase > 11.4 ng/mL in peripheral blood is the principal biochemical marker for anaphylaxis...
- SIDS (Sudden Infant Death Syndrome)
- A diagnosis of exclusion under the AAP definition: sudden unexpected death of an infant under one year, occurring apparently during sleep, unexplained...
- Sudden Cardiac Death (SCD)
- Natural unexpected death from a cardiac cause within one hour of symptom onset, without a prior condition that would appear immediately fatal....
- Triple-Risk Model (SIDS)
- The Filiano and Kinney 1994 model proposing that SIDS deaths occur at the intersection of a vulnerable infant (brainstem arousal-response immaturity), a...
Explained in
- Sudden Natural Deaths: Cardiac, SIDS, AnaphylaxisCardiomyopathy in which right ventricular myocardium is progressively replaced by fibrofatty tissue, predisposing to right ventricular arrhythmia and sudden de...