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 (e;g;stop

alcohol )
2. Threat cardiac failure ( diuretics, ACE inhibitors,
nitrates )
3. Therat any arrhytmias ( digoxin or amiodarone for
atrial fibrillation, amiodarone for ventricular
arrhytmias )
4. 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.
 β-Blockers (β-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 end-
diastolic 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 Kussmaul’s 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