Cardiomyopathy

Prof. dr. Harris Hasan SpPD,SpJP(K),FIHA

Departemen Kardiologi & Kedokteran Vaskular
FK USU RSUP H.Adam Malik







Definition of cardiomyopathy
Cardiomyopathy is heart muscle disease,
often of unknown cause. There are three
types
1. Dilated cardiomyopathy
2. Hypertropic obstructive cardiomyopathy
(HOCM)
3. Restrictive cardiomyopathy
Dilated Cardiomyopathy
The heart is dilated and has impaired function.
The coronary arteries are normal. Probable
causes of dilated cardiomyopathy include :
Alcohol
Viral infection (echovirus, coxsackievirus, and
enteroviruses most likely)
Untreated hypertension
Autoimmune disease
Thyrotoxicosis
Drugs (cocaine, doxorubicin, cyclophosphamide,
lead)
Haemochromatosis
Acquired immune deficiency syndrome (AIDS)
Clinical features
Progressive biventricular cardiac failure leads to :
Fatigue
Dyspnoea
Peripheral oedema
Ascites

Other complications secondary to the
progressive dilatation of the ventricles include :
Mural thrombi with systemic or pulmonary
embolization
Dilatation of the tricuspid and mitral valve rings
leading to functional valve regurgitation
Atrial fibrillation
Ventricular tachyarrhthmias and sudden death
Investigation
Chest radiography
This may show :
Enlarged cardiac shadow
Signs of pulmonary oedema (upper lobe blood
diversion, interstitial shadowing at the bases)
Pleural effusions

Electrocardiography
Electrocardiography may highlight :
Tachycardia
Poor R wave progression across the chest leads
Echocardiography
Points to consider with echocardigraphy include :
Can the dilated ventricles be easily visualized?
Can the regurgitant valves be seen?
Occasionally, intracardiac thrombus may be seen.
Transthoracic echocardiography is not a reliable method
for diagnosing this but it can be accurately diagnosed by
transoesophageal echocardiography.

Cardiac catheterization
This is important to exclude coronary artery disease (the
most common cause of ventricular dysfunction).

Blood tests
Viral titres may be 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 treat any underlying cause (e.g.stop
alcohol)
2. Treat cardiac failure (diuretics, ACE inhibitors, nitrates)
3. Treat any arrhythmias (digoxin or amiodarone for atrial
fibrillation, amiodarone for ventricular arrhythmias)
4. Anticoagulate with warfarin to prevent mural thrombi.

If the cardiac failure does not respond to the above
steps and the patient is suitable candidate then cardiac
transplantation is a possible option.
Hypertrophic obstructive
cardiomyopathy
This disorder is characterized by asymmetrical
hypertrophy of the cardiac septum-the cardiac
septum is hypertrophied compared to the free
wall of the left ventricle.
Hypertrophic obstructive cardiomyopathy is
inherited as an aotusomally dominant trait with
equal sex incidence.
The myocytes of the left ventricle are
abnormally thick when examined
microscopically.

65% 35%
10%
www.kanter.com/hcm
Clinical features

There are four main symptoms :
1. Angina (even in the absence of coronary artery
disease)-due to the increased oxygen demands of the
hypertrophied muscle
2. Palpitations-there is an increased incidence of atrial
fibrillation and ventricular arrhythmias in this condition
3. Syncope and sudden death-which may be due to left
ventricular outflow tract obstruction by the
hypertrophied septum or to a ventricular arrhythmia
4. Dyspnoea-due to the stiff left ventricle, which leads to
a high end-diastolic pressure and can therefore lead to
pulmonary oedema.

It can be difficult to differentiate between HOCM
and aortic stenosis on examination. Use the
following features to help :
Pulse slow rising in aortic stenosis, jerky or
with a normal upstroke in HOCM.
Thrill and murmur both found in the second
right intercostal space in aortic stenosis and at
the left lower sternal edge in HOCM.
Variation of the murmur with Valsava manoeuvre
this does not occur in aortic stenosis, but the
murmur of HOCM is increased because the
volume of the left ventricle is reduced by the
manoeuvre and therefore the outflow obstruction
worsens.

Diagnosis and investigations

Electrocardiography
This is usually abnormal in HOCM. The most
common abnormalities are T wave and ST
segment abnormalities; the signs of left
ventricular hypertrophy may also be present.


Continuous ambulatory electrocardiography

The presence of ventricular arrhythmias is
common in patients who have HOCM and is a
cause of sudden death. It is thought that the
presence of ventricular arrhythmias on an
ambulatory ECG monitor is a risk factor for
sudden death and that an antiarrhythmic agent
should be commenced. These tests are usually
performed as part of a yearly screening
programme for these patients.

Echocardiography
This is the most useful investigation because it
confirms the diagnosis and can be used to
assess the degree of outflow tract obstruction.
Characteristic echocardiography findings
include :
Asymmetrical hypertrophy of the septum.
Abnormal systolic anterior motion of the anterior
leaflet of the mitral valve.
Left ventricular outflow tract obstrustion.


Prognosis

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

Management
Drug 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 nitrates.
-blockers (-adrenoceptor antagonists) are
used because their negative inotropic effect acts
to decrease the contractility of the hypertrophied
septum and reduce the outflow tract obstruction.
Antiarrhythmic agents are important in patients
who have ventricular and atrial arrhythmias.
Dual-chamber pacing

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


Surgery

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

Restrictive cardiomyopathy
This is the least common of the cardiomyopathies in
developed countries. The ventricular walls are
excessively stiff and impede ventricular filling, therefore
end-diastolic pressure is increased. The systolic function
of the ventricle is increased.
Possible causes of restrictive cardiomyopathy include :
Storage diseases (e.g.glycogen storage diseases)
Infiltrative diseases (e.g.amyloidosis, sarcoidosis)
Scleroderma
Endomyocardial diseases (e.g.endomyocardial fibrosis,
hypereosinophilic syndrome, carcinoid)


Clinical features

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

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