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Cardiomyopathies
Hypertrophic cardiomyopathy
The condition was first recognised in the 1950's and has been known by the names Hypertrophic Obstructive Cardiomyopathy (HOCM), Idiopathic Hypertrophic Sub-aortic Stenosis (IHSS) and Muscular Sub-aortic Stenosis but the term Hypertrophic Cardiomyopathy (HCM) is now generally used.
HCM is an inherited condition which, in the majority of affected people, does not limit the quality and duration of life. It is a disorder of the heart consisting of excessive growth of the muscle, which may begin before birth when the fetal heart is developing. The natural history is characterised by slow progression of symptoms but a significant incidence of sudden death. Symptoms of chest pain, dizziness, loss of consciousness, palpitation and breathlessness can occur but some affected persons do not experience symptoms. Irregularity of the heartbeat can lead to palpitations but usually is only detected during ECG monitoring. Treatment is usually by drug therapy but surgery may be indicated where the thickening of the muscle causes obstruction to the outflow tract of the heart. Risk stratification to identify an affected individual's potential risk of sudden death is an important part of medical management to ensure appropriate preventative measures are taken. If the condition is diagnosed, intense competitive exercise should be avoided. Special monitoring during anaesthesia is required.
If the condition is diagnosed, violent exercise, prolonged standing in hot conditions and very hot baths/showers should be avoided. Special monitoring during anaesthesia is required.
Inheritance patterns
Autosomal dominant. Family screening is strongly recommended. Research shows 1 in 500 people are affected in the UK
Prenatal diagnosis
None.
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