100 QUESTIONS IN CARDIOLOGY

(Michael S) #1

48 How should I investigate a patient with


hypertrophic cardiomyopathy (HCM)?


Krishna Prasad


The first step in the investigation of a patient with hypertrophic

cardiomyopathy (HCM) is to establish the diagnosis and determine

whether the case is sporadic or familial.

History


The investigation should begin with the taking of a detailed history.

This should include the construction of a family tree with at least

three generations.

The clinical examination


This should be aimed specifically at excluding other causes of

hypertrophy such as aortic stenosis or hypertension.

Descriptive investigations


Electrocardiography. In the majority of patients, the 12-lead electro-

cardiogram (ECG) shows abnormalities such as voltage criteria

for left ventricular hypertrophy (LVH), minor intraventricular

conduction defects or bundle branch blocks. Only rarely (<5% of

cases) is the ECG completely normal.

Echocardiography. The mainstay of diagnosis is the echocardio-

graphic demonstration of left ventricular hypertrophy (LVH),

with either the interventricular septum or the free wall measuring

15mm. A very detailed study by an experienced operator is

often necessary as hypertrophy may involve any part of LV. It is

important to note that for adults with family history of HCM,

modified diagnostic criteria apply.

Investigations to identify risk factors of sudden death


The recognised risk factors are family history of sudden deaths,

recurrent syncope, non-sustained ventricular tachycardia and an
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