CARDIOMYOPATHY

Definition of cardiomyopathy
Cardiomyopathy is heart muscle disease, often
of unknown cause. There are three types
1. Dilated Cardiomyopathy
2. Hypertrophic Obstructive Cardiomyopathy
3. Restrictive Cardiomyopathy

Dilated cardiomyopathy
The heart is dilated and has impaired function.
The coronary arteries are normal. esable
causes of dilated cardiomyopathy included
-

Alcohol
Viral infection (Probchovirus, coxsackievirus, and
enterovirusmost likely )
Untreated hypertension
Autoimmune disease
Thyrotoxicosis
Drugs
(
caocaine,
doxorubicin,
cylcophosphamide, lead )
Haemochromatosis
Acquired immune deficiency syndrome ( AIDS )

Clinical Features
Progeressive biventricular cardiac failure
leads to :
1. Fatigue
2. Dyspnoea
3. Peripheral oedema
4. Ascites

Other complications secondary to the

progressive dilatation of the ventricles
include :
- Mural thrombi with systhemic or
pulmonary embolization
- Dilatation of the triscupid and mitral valve
rings leading to functional valve
regurgitation
- Atrial fibrillation
- Ventricular tachyarrhytmias and sudden
death

Investigations
Investigations to aid diagnosis are
listed below

Chest Radiography
This may show
- Enlarge cardiac shadow
- Signs pulmonary oedema (upper lobe
blood diversion, interstitial
shadowing at the bases )
- Pleural effusions

Electrocardiography
Electrocardiomay highlight :

Tachycardia
Poor R wave progressions across
the chest leads

Echocardiography
Points to consider with ecocardiography include :
1. Can the dilated ventricles be easily visualized
?
2. Can the regurgitant valves be seen ?
Occasionally, intracardiac thrombus may be
seen. Transthoracic ecocardiography is not a
reliable method for diagnosing this but it can
be accurately diagnosed by
transoesophageal echocardiography.

Cardiac actheterization
This is important to exclude coronary
artery disease ( the most common
cause of ventricular dysfunction )
Blood test
Viral titres may e useful and also
thyroid function tests

Management
The management plan follows four basic steps (
the same applies for any other case of cardiac
failure ) :

1. Search for and threat any underlying cause

2.
3.
4.

(e;g;stop alcohol )
Threat cardiac failure ( diuretics, ACE inhibitors,
nitrates )
Therat any arrhytmias ( digoxin or amiodarone
for atrial fibrillation, amiodarone for ventricular
arrhytmias )
Anticoagulate warfarin to prevent mural thrombi

Hypertrophic obstructive
cardiomyopathy
This disorder is characterized by asymmetrical
hypertrophy of cardiac septum, the cardiac septum is
hypertrophied compared to the free wall of the left
ventricle.
Hypertrophic obstructive cardiomyopathy is inherited as
an autosomally dominant trait with equal sex incidence.
The genetic abnormally is the subject of much current
research and it seems that different genes may be
involved in different families.
The miocytes of the left ventricle are abnormally thick
when examined microscopically. This maks left
ventricular filling more difficult than normal and grossly
disordered

Clinical features
There are four main symptoms :
1. Angina
2. Palpitations
3. Syncope and sudden death
4. Dyspnoea

DIAGNOSIS AND
INVESTIGATIONS

Electrocardiography
This is usually abnormal in HOCM. The most common
abnormalities are T wave and ST segment
abnormalities, the signs ofleft ventricular hipertrophy
may also be present
Continous ambulatory electrocardiography
The presence of ventricular arrhytmias is common in
patients who have HOCM and is cause ofsudden death.
It is thought that the presence of ventricular
arrhytmiass\ on an ambulatory ECG monitor is a risk
for factor of sudden death and that an antiarrhythmic
agent should be commenced. These tests are usually
performed as part of a yearly screening programme for
the patients

Echocardiography
This is most useful investigation
because it comfirm of diagnosis and
can be used to assess the degree of
outflow tract obstruction
Characteristic echocardiography
findings include :
- Assymmetrical hypertrophy of the
septum
- Abnormal systolic anterior motion of
theanterior leaflet of yhe mitral valve
- Left venricular out flow tract obstruction

Prognosis
Childern who are diagnosed when they
are under 14 years of age a poor
prognosis and a high incidence of
sudden death. Adults have a better
prognosis, but they are also have a
higher mortality rate than the general
population. Another outcome is
progressive cardiac failure with cardiac
dilatation.

MANAGEMENT
Drugs management
As with aortic stenosis, vasodilators
should be avoided because they
worsen the gradient across the
obstruction. Therefore patients who
have HOCM should not receive nitrate.

-Blockers (-adrenoceptor antagonists )

are used because their negative inotropic
effect acts to decrease the contractility of the
hyperthropeid septum and reduce the outflow
tract obstruction
Antiarrhythmic agents are important in
patient swho have ventricular and atrial
arrhythmias. Patients who have arial
fibrillation should be cardioverted as soon as
possible( patients who havea high left
ventricular end-diastolic pressure rely on the
atrial impulse to fill the left ventricle
effectively )

Dual chamber pacing

This reduce the outflow tract gradient by
pacing heart from the right ventricular
apex and therefore altering the patern of
septal motion. New devices incorporate
both adual-chamber pacemaker and a
cardioverter defebrillator to treat
malignant ventricular arrhythmias in
patients with HOCM

Surgery
Surgery is used only when all the other
treatments have failed. A
myomectomy is performed on the
abnormal septum. There is a new
catheter tehnique to infarct the
septum by occuding the septal artery

Retrictive Cardiomiopathy
This is the least common of the
cardiomyopathies in developed countries.
The entricular walls are excissevely enddiastolic pressure is increased. The systolic
function of the ventricle is often normal

Possible cause of restrictive

cardiomyopathy

Storage diseases ( e.g. glycogen storage

disease )
Infiltrative diseases ( e.g. amyloidosis,
sarcoidosis)
Scleroderma
Endomyocardial diseases ( e.g.
endomyocardial fibrosis, hypereosinophilic
syndrome,
carcionoid )

Clinical Features
The main features are :
Dyspnoea and fatigue due to poor
cardiac output
Peripheral oedema and ascites
Elevated jugular venous pressure
with a positive Kussmauls sign
( increase in jugular venous
pressure during inspiration )

Management
There is no spesific treatment and the
condition. Usually progresses towards
death relatively quickly ; most patients
do not survive beyond 10 years after
diagnosis