100 QUESTIONS IN CARDIOLOGY

(Michael S) #1
Hence first degree relatives should undergo history, physical

examination, standard 2-D echocardiography, and 12-lead

electrocardiography. Relatives are considered affected in the

presence of one major criterion or two minor echocardiographic

criteria or one minor echocardiographic plus two minor electro-

cardiographic criteria. These criteria do not apply when other

potential causes such as athletic training, systemic arterial hyper-

tension or obesity are present. Young children with no evidence

of disease should be re-evaluated every 5 years until their teens

and then annually until aged 21. Diagnosis in a child under 10

years requires a body surface area corrected left ventricular wall

thickness of >10mm. Affected relatives should additionally

undergo risk stratification, which includes 48 hour Holter

monitoring and exercise testing, looking especially for

ventricular arrhythmias and abnormal blood pressure responses

respectively.

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McKenna WJ, Spirito P, Desnos M et al. Experience from clinical genetics
in hypertrophic cardiomyopathy. Proposal for new diagnostic criteria in
adult members of affected families. Heart1997; 7777 : 130–2.

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