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Chronic Care Programme
Treatment guidelines
Cardiomyopathy
Heart Muscle Disease
Chronic condition
Alternative names
Consultations protocols
Preferred treating provider
Notes
 preferred as indicated by option
 referral protocols apply
Provider
General Practitioner
Physician
Paediatrician
Cardiologist
Cardiology Paediatrician
Surgeon
Thoracic Surgeon
New Patient
Maximum consultations per annum
 Initial consultation
 Follow-up consultation
Tariff codes
Option/plan
GMHPP
Gold Options
G1000, G500 and
G200.
Blue Options
B300 and B200.
GMISHPP
Existing patient
Stable
Unstable
Controlled
Uncontrolled
1
1
3
1
0183; 0142; 0187; 0108
1
5
Investigations protocols
Type
Provider
Treating
doctor
Urine dipstick (per stick,
irrespective of the number of
tests on stick)
Blood glucose:
quantitative
Blood glucose: strip test with
Pathologist
or as per
treating
doctor
Pathologist
or as per
treating
doctor
Any
Maximum investigations per annum
Mild
Moderate to severe
1) Patient with cardiac disease but 1) Patient comfortable at
no limitation on ordinary
rest, less than ordinary
physical activity eg can climb
activity results in
stairs / walk uphill without
symptoms (marked
symptoms.
limitation) eg can walk <
2) Patient comfortable at rest,
1 – 2 blocks: can climb <
ordinary activity results in
1 flight of stairs:
symptoms (slight limitation) eg
intolerant of walking
can walk > 2 block on level
uphill
ground: can climb 1 flight of
stairs at normal pace:
2) Patient symptomatic at
symptomatic on walking uphill.
rest, increases discomfort
with any physical activity
New
Existing
New & Existing patient
patient
Patient
Tariff
code
4188
4
2
4
4057
1
1
1
photometric reading
ECG without effort
ECG without and with effort
Multistage treadmill test
Cardiac examination:
(M mode)
Cardiac examination:
2 dimensional
Cardiac examination +
Doppler: Add
Haemoglobin estimation
Packed cell volume:
Haematocrit
Platelet count
Chol / HDL / LDL / Trig
Serum urea
Serum creatinine
Serum potassium
Serum sodium
Digoxin level (Drug level –
biological fluid: quantitative)
Digoxin level (Drug
concentration: quantitative)
Gamma glutamyl transferase
(ggt)
TSH
Protein: total
Aspartate aminotransferase
(AST)
Alanine aminotransferase
(ALT)
Lactate dehydrogenase (LD)
Albumin
INR / Pro-thrombin index
CXR
ICD 10 coding
preferred
provider
Any
preferred
provider
General
Practitioner
Specialists
(see list)
Specialists
(see list)
Specialists
(see list)
4050
1
1
1
1232;
1233
1
1
2
1233
1
1
1
1235
1
1
1
3621
1
0
1
3622
1
1
1
Specialist
(see list)
Pathologist
or as per
treating
doctor
Pathologist
3625
1
1
2
3762
1
1
2
3791
1
1
2
Pathologist
Pathologist
Pathologist
Pathologist
Pathologist
Pathologist
Pathologist
3797
4025
4151
4032
4113
4114
4081
1
1
1
1
3
1
0
1
1
1
1
2
1
0
2
1
2
2
4
2
3
Pathologist
4493
0
0
3
Pathologist
4134
1
0
1
Pathologist
Pathologist
Pathologist
4507
4117
4130
1
0
0
0
0
0
1
1
1
Pathologist
4131
0
0
1
Pathologist
Pathologist
Pathologist
Radiologist
4133
3999
3805
3445
0
0
0
1
0
0
0
1
1
1
6
2
I42.-I43.
General
Cardiomyopathy, which literally means "heart muscle disease", is the deterioration of the
function of the myocardium (i.e., the actual heart muscle) for any reason. People with
cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both.[1]
Classification
Cardiomyopathies can generally be categorized into two groups, based on World Health
Organization guidelines: extrinsic cardiomyopathies and intrinsic cardiomyopathies.[2]
Extrinsic cardiomyopathies
These are cardiomyopathies where the primary pathology is outside the myocardium itself. Most
cardiomyopathies are extrinsic, because by far the most common cause of a cardiomyopathy is
ischemia. The World Health Organization calls these specific cardiomyopathies:[2]




Coronary artery disease
Congenital heart disease
Nutritional diseases
Ischemic (or ischaemic) cardiomyopathy



Hypertensive cardiomyopathy
Valvular cardiomyopathy
Inflammatory cardiomyopathy


Cardiomyopathy secondary to a systemic metabolic disease
Alcoholic cardiomyopathy
Ischemic cardiomyopathy
Ischemic cardiomyopathy is a weakness in the muscle of the heart due to inadequate oxygen
delivery to the myocardium with coronary artery disease being the most common cause. Anemia
and sleep apnea are relatively common conditions that can contribute to ischemic myocardium
and hyperthyroidism can cause a 'relative' ischemia secondary to high output heart failure.
Individuals with ischemic cardiomyopathy typically have a history of myocardial infarction (heart
attack), although longstanding ischemia can cause enough damage to the myocardium to
precipitate a clinically significant cardiomyopathy even in the absence of myocardial infarction.
In a typical presentation, the area of the heart affected by a myocardial infarction will initially
become necrotic as it dies, and will then be replaced by scar tissue (fibrosis). This fibrotic tissue
is akinetic; it is no longer muscle and cannot contribute to the heart's function as a pump. If the
akinetic region of the heart is substantial enough, the affected side of the heart (i.e. the left or
right side) will go into failure, and this failure is the functional result of an ischemic
cardiomyopathy.
Cardiomyopathy due to systemic diseases
Many diseases can result in cardiomyopathy. These include diseases like hemochromatosis, (an
abnormal accumulation of iron in the liver and other organs), amyloidosis (an abnormal
accumulation of the amyloid protein), diabetes, hyperthyroidism, lysosomal storage diseases and
the muscular dystrophies.
Intrinsic cardiomyopathies
An intrinsic cardiomyopathy is weakness in the muscle of the heart that is not due to an
identifiable external cause. To make a diagnosis of an intrinsic cardiomyopathy, significant
coronary artery disease should be ruled out (amongst other things). The term intrinsic
cardiomyopathy does not describe the specific etiology of weakened heart muscle. The intrinsic
cardiomyopathies are a mixed-bag of disease states, each with their own causes.
Intrinsic cardiomyopathy has a number of causes including drug and alcohol toxicity, certain
infections (including Hepatitis C), and various genetic and idiopathic (i.e., unknown) causes.
Intrinsic cardiomyopathies are generally classified into four types,[2][3] but additional types are
also recognized:

Dilated cardiomyopathy (DCM), the most common form, and one of the leading
indications for heart transplantation. In DCM the heart (especially the left ventricle) is
enlarged and the pumping function is diminished. Approximately 40% of cases are
familial, but the genetics are poorly understood compared with HCM. In some cases it
manifests as peripartum cardiomyopathy, and in other cases it may be associated with
alcoholism.

Hypertrophic cardiomyopathy (HCM or HOCM), a genetic disorder caused by various
mutations in genes encoding sarcomeric proteins. In HCM the heart muscle is thickened,
which can obstruct blood flow and prevent the heart from functioning properly.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) arises from an electrical
disturbance of the heart in which heart muscle is replaced by fibrous scar tissue. The right
ventricle is generally most affected.

Restrictive cardiomyopathy (RCM) is an uncommon cardiomyopathy. The walls of the
ventricles are stiff, but may not be thickened, and resist the normal filling of the heart with
blood. A rare form of restrictive cardiomyopathy is the obliterative cardiomyopathy, seen
in the hypereosinophilic syndrome. In this type of cardiomyopathy, the myocardium in the
apices of the left and right ventricles becomes thickened and fibrotic, causing a decrease
in the volumes of the ventricles and a type of restrictive cardiomyopathy.

Noncompaction cardiomyopathy has been recognized as a separate type since the 1980s.
The term refers to a cardiomyopathy where the left ventricle wall has failed to grow
properly from birth and has a spongy appearance when viewed during an echocardiogram.
Signs and symptoms
Signs and symptoms Cardiomyopathy is usually found incidentally - "case finding" - by
healthcare professionals during a routine checkup. The only test for hypertension is a blood
pressure measurement. Hypertension in isolation usually produces no symptoms although some
people report headaches, fatigue, wanting to sleep more than usual, dizziness, blurred vision,
facial flushing or tinnitus. [10]
Malignant Cardiomyopathy (or accelerated Cardiomyopathy) is distinct as a late phase in the
condition, and may present with headaches, blurred vision and end-organ damage.
Cardiomyopathy is often confused with mental tension, stress and anxiety. While chronic anxiety
and/or irritability is associated with poor outcomes in people with hypertension, it alone does not
cause it. Accelerated hypertension is associated with somnolence, confusion, visual disturbances,
and nausea and vomiting (hypertensive encephalopathy)
Diagnosis
The diagnosis is based on the patient’s symptoms, a complete physical examination, and tests that
detect abnormalities of the heart chambers. The physician listens to the heart with a stethoscope
to detect abnormal heart rhythms and heart sounds. A heart murmur might mean that the heart
valves are not closing properly due tothe ventricles being enlarged. A chest x ray can show if the
heart is enlarged and if there is fluid in the lungs. Abnormalities of heart valves and other
structures may also be seen on a chest x ray. An electrocardiogram provides a record of electrical
changes in the heart muscle during the heartbeat. It gives information on the heart rhythm and can
show if the heart chamber is enlarged. An ECG can detect damage to the heart muscle and the
amount of damage.
Echocardiography uses sound waves to make images of the heart. These images can show if the
heart wall or chambers are enlarged and if there are any abnormalities of the heart valves.
Echocardiography can also evaluate the pumping efficiency of the ventricles.
Cardiac catheterization usually is only used if a diagnosis cannot be made with other methods.
In cardiac catheterization, a small tube (called a catheter) is inserted into an artery and passed into
the heart. It is used to measure pressure in the heart and the amount of blood pumped by the heart.
A small tissue sample of the heart muscle can be removed through the catheter for examination
under a microscope (biopsy). This biopsy can show the type and amount of damage to the heart
muscle
Treatment
Treatment depends on the type of cardiomyopathy, but may include medication, implanted
pacemakers, defibrillators, or ventricular assist devices (LVADs), or ablation. The goal of
treatment is often symptom relief, and some patients may eventually require a heart transplant.
Treatment of cardiomyopathy (and other heart diseases) using alternative methods such as stem
cell therapy is commercially available but is not supported by convincing evidence.
Medicine formularies
Plan or option
GMHPP
Gold Options
G1000, G500 and
G200
Blue Options
B300 and B200
GMISHPP
Blue Option 100
Link to appropriate Mediscor formulary
[Core]
n/a
Epidemiology
About 50,000 Americans develop cardiomyopathy each year. Of those, 87% have congestive
cardiomyopathy. Primary cardiomyopathy accounts for only 1% of all
deaths from heart disease
Prognosis
Prognosis
The outlook for a patient with congestive cardiomyopathy depends on the severity of the disease
and the person’s health. Generally, congestive cardiomyopathy worsens over time and the
prognosis is not good. About 50% of patients with congestive cardiomyopathy live for
five years after the diagnosis. Twenty five percent of patients are alive 10 years after diagnosis.
Women with congestive cardiomyopathy live twice as long as men with the disease. Many of the
deaths are caused by sudden abnormal heart rhythms.
Prevention
Because idiopathic congestive cardiomyopathy does not have a known cause, there is no sure way
to prevent it. The best way to prevent congestive cardiomyopathy is to avoid known causes such
as drinking excess alcohol or taking toxic drugs. Eating a nutritious diet and getting
regular exercise to improve overall fitness also can help the heart to stay healthy.
Congestive cardiomyopathy may also be prevented by
identifying and treating any conditions that might damage the heart muscle. These include high
blood pressure and coronary artery disease. Regular blood pressure checks and obtaining
immediate medical care for hypertension and symptoms of coronary artery disease, such as chest
pain, are important to keep the heart functioning properly.
Finally, diagnosing and treating congestive cardiomyopathy before the heart becomes severely
damaged may improve the outlook.
References
1. Kasper, Denis L. et al (2005). Harrison's Principles of Internal Medicine, 16th edn.
McGraw-Hill. ISBN 0-07-139140-1.
2.
abc
Richardson, P. et al (1996). "Report of the 1995 World Health
Organization/International Society and Federation of Cardiology Task Force on the
Definition and Classification of cardiomyopathies". Circulation 93 (5): 841-2. PMID
8598070. (Full text)
3. Cardiomyopathy Association. About cardiomyopathy. Retrieved on 2006-09-28.
External links

The Cardiomyopathy Association A site is designed to provide you with information on
the main forms of the heart muscle disease known as cardiomyopathy. Great information




for adults and young.
Cardiomyopathy information from Seattle Children's Hospital Heart Center
Information from the Stanford Hypertrophic Cardiomyopathy Center
Cardiomyopathy-related antibodies
Treatment Cardiomyopathy (Alternative Medicine)