Hypertrophic Cardiomyopathy (HCM)
Definition
Autosomal dominant structural cardiomyopathy characterised by asymmetric septal hypertrophy (septal:free-wall thickness ratio > 1.3) and microscopic myocyte disarray covering > 5 percent of the ventricular cross-section. Leading structural cause of sudden cardiac death in young athletes in the UK, Italy, and US autopsy series.
Related terms
- ARVC (Arrhythmogenic Right Ventricular Cardiomyopathy)
- Cardiomyopathy in which right ventricular myocardium is progressively replaced by fibrofatty tissue, predisposing to right ventricular arrhythmia and sudden death. Leading cause...
- Channelopathy
- Ion-channel gene mutation causing potentially fatal arrhythmia without structural cardiac abnormality. The three principal forensic channelopathies are long QT syndrome (LQTS), Brugada...
- 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, AnaphylaxisAutosomal dominant structural cardiomyopathy characterised by asymmetric septal hypertrophy (septal:free-wall thickness ratio > 1.3) and microscopic myocyte di...