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Abdullah Saadah
Hala Al Khateeb
18 / 02 / 2010

Some notes before you begin:

The recorder isnt for the whole lecture; the last four topics are not covered.

There is extra information in ru2ia lecture, I tried to include some of this

information, and it is in boxes with dashed line.

Extra information from the internet is in boxes with triple line.

Cardiomyopathy is the 3rd most common form of heart disease in U.S. and the 2nd most
common cause of adolescent sudden death in the forms of (Idiopathic hypertrophic
subaortic stenosis (IHSS) or Hypertrophic obstructive Cardiomyopathy (HOCM).It
directly affects the cardiac structure and impairs the myocardial function.

It is divided mainly into four types:

Dilated Cardiomyopathy (DCM) which is the most common type.
Hypertrophied Cardiomyopathy (HOCM).
Restrictive Cardiomyopathy.
Dysrhythmic right ventricular cardiomyopathy.

Dilated Cardiomyopathy:
In which we have dilation and compensatory hypertrophy of the myocardium, this causes
depressed systolic function and pump failure with low cardiac output.

80% of DCM cases are idiopathic

chronic alcohol intake

In multiparous woman (woman with multiple pregnancies) and the incidence

increases as the number of pregnancies increases.

Drug induced and mainly with doxorubicin which is an anti-neoplastic


African Americans and males have 2.5 times increased risk. And the most common age of
diagnosis 20-50yrs.
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Clinical Presentation:
Typical Signs and symptoms of congestive heart failure (CHF); dyspnea on exertion,
orthopnea and PND (paroxysmal nocturnal dyspnea), lower limb edema, hepatic
Chest pain can occur due to low perfusion and low coronary vascular reserve;
because of the dilation of the myocardium, the coronary tree covers larger area to
supply, which causes less blood supply to that area and chest pain.
Because of the low cardiac output and because of the dilation, stagnation of the
blood in the left ventricle will occur and Mural thrombi can form, this may cause
peripheral vascular disease, renal shutdown or even CVA.
Holosystolic regurgitant murmur or gallop may
be present because of the dilation there is
stretching of the mitral valve of the papillary
muscles of the chorda tympani and the cups of
the mitral valve will not close well during systole,
this is called functional mitral valve disease and
has no treatment.

bibasilar rales:
Abnormal breath sounds (crackles)
heard on auscultation only in the
bases of the lungs. They indicate
inflammation, fluid, or infection in
the air sacs of the lung.

Dependent edema (lower limb edema).

Bibasilar rales( crepitations in both lungs).
So it is a combination of left sided heart failure leaded to right ventricle
heart failure.

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We mainly do the diagnosis depending on the clinical picture; clinically the pt. with dilated
cardiomyopathy shows stigmata of CHF.
Chest X ray : we will find enlarged heartcardiomegaly, biventricular enlargement,
increase in the pulmonary vascular marking with congestion(cephalization) and upward
diversion of the pulmonary vessels in the upper lobes indicating pulmonary edema,
hilar engorgement and increase in the hilar marking

which are all signs of heart

1) LVH
2) Left atrial enlargement determined by the negativity of the P wave in lead I
increases and in lead II shows sudden P wave.
3) Q waves,
4) poor R wave progression in lead (I-III),
5) Any type of arrhythmia can occur but especially atrial fibrillation and
sometimes sinus tachycardia; with the enlargement of the heart the action
potential travel longer distance because when the myocytes get enlarged the
conductance system doesnt enlarge with them and thats why part of the action
potential will be conducted through the myocardial muscle itself, and this action
potential will be transmitted slower and causes slow beat, on the ECG shows
wider QRS than normal, the wider the QRS in the patient with dilated
cardiomyopathy the worse the prognosis.
Echo-Confirms the diagnosis and shows: dilation of the left ventricle, decreased
ejection fraction that may reach 30, enlargement in the right ventricle can be
seen later on and thats because the rise in the left ventricular systolic pressure
causes increase in the left atrial pressure which causes left atrial enlargement and
causes pulmonary edema or pulmonary hypertension, then right atrial pressure
increases and finally the right ventricle pressure will increase causing right ventricle
enlargement and failure, and as a consequence of the right ventricle failure we will
have congestion in the systemic circulation manifested by ascites and lower limb
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Differential diagnosis:
Acute MI: Clinically dilated cardiomyopathy comes with the stigmata of congestive
heart failure and the cardiac echo shows that the left ventricle is enlarged, so it is
hardly to differentiate between ischemic cardiomyopathy and dilated
cardiomyopathy, so if the patient came with the history of coronary artery disease
then it is ischemic cardiomyopathy while if he came with dilated cardiomyopathy it
is usually idiopathic.
Restrictive Pericarditis it differs from dilated cardiomyopathy by the echo; in the
echo Restrictive Pericarditis shows small ventricle compared to the dilated
cardiomyopathy where it shows large left ventricle also the thickness of the
myocardium is more in Restrictive Pericarditis than that it is in the dilated
Acute valvular disruption usually causes acute re-compensation and acute heart
failure; it is usually easy by echo to differentiate between vulular disruption that
is functional rather than organic.
Any form of hypotension that results in low cardiac output state can come in the
differential of dilated cardiomyopathy.

ED care and disposition

Newly diagnosed or symptomatic DCM and come to the ER with decompensated heart
failure of course the treatment is composed of:
put on O2.
IV lazix improves symptoms, decreases the end diastolic pressure and decreases
the lung congestion.
ACE inhibitors and B-Blockers-improve survival but not to improve survival in
patients with dilated cardiomyopathy secondary to ischemic heart disease.

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B-BLOCKERS are imp in cardiomyopathies in general and also imp to patients with
heart failure, B-blocker tend to decrease the tendency for complex arrhythmia
and the risk for sudden death. But only some types of b-blocker are indicated in
de-compensated heart failure (Bisoprolol, Metoprolol, and Carvedilol)
Anticoagulation may be considered usually in pts with dilated cardiomyopathy,
there is a rule that if the ejection fraction is less than 30 administer
anticoagulation therapy, but you should consider every patient as a separate case
and consider what other conditions the patient.
Warfarin is an anti coagulation drug but with a low therapeutic index which means that
the therapeutic dose is little less than the toxic dose, usually warfarin is indicated for
whom with an ejection fraction less than 30 or who are susceptible to mural thrombi

Hypertrophic Cardiomyopathy:
Sometimes its called idiopathic hypertrophic
subaortic stenosis (IHSS) in which the hyper trophy
happens in the outflow tract, hypertrophy can
happen in different parts of the left ventricle but
the most famous type is Usually in the out flow
tract, sometimes there is septal (posterior wall)
hypertrophy and sometimes there is apical

The second most common form of

heart muscle disease is hypertrophic
cardiomyopathy . Physicians
sometimes call it by other names:
idiopathic hypertrophic subaortic
stenosis (IHSS), asymmetrical septal
hypertrophy (ASH), or hypertrophic
obstructive cardiomyopathy (HOCM).

hypertrophy. Not all hypertrophic

cardiomyopathies can present with obstruction,


only 40 % of HOCM presents with obstructive

Dynamic outflow obstruction (when

present in HCM) is usually due to systolic
anterior motion (SAM) of the anterior
leaflet of the mitral valve. Systolic anterior
motion of the mitral valve (SAM) was
initially thought to be due to the septal
subaortic bulge, narrowing the outflow
tract, causing high velocity flow and
a Venturi effecta local underpressure in
the outflow tract.


non-obstructive variant of HCM

is apical hypertrophic

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Asymmetrical LVH and/or RVH, primarily it

involves the septum, rarely we can find
only right ventricular hypertrophy. Usually
without dilation except for the end stage
of the disease when the left ventricle

In hypertrophic cardiomyopathy, the

growth and arrangement of muscle
fibers are abnormal, leading to
thickened heart walls. The greatest
thickening tends to occur in the left


ventricle (the heart's main pumping

It leads to abnormal compliance, impaired

wall that separates the left and right

diastolic relaxation but filling-output

ventricles. The thickening reduces the

usually are normal, in dilated

size of the pumping chamber and

cardiomyopathy we said that the type of

obstructs blood flow. It also prevents

the heart failure is systolic type of heart

the heart from properly relaxing

failure while in HCM its a diastolic type of

between beats and so filling with blood.

heart failure. It is a failure of the left

Eventually, this limits the pumping

ventricle to accept the blood pumped by


chamber), especially in the septum , the

the left atrium, rather than the blood

lagging in the ventricle in the case of dilated cardiomyopathy. so it is called
impaired diastolic relaxation but usually the cardiac output is normal.
50% are hereditary.
Prevalence 1 in 500, Mortality 1%
Mortality 4-6% in childhood/adolescence, because when it happens in the young it
carries worse prognosis.

Clinical Features
Sometimes its difficult to differentiate on the clinical examination between congestive
heart failure due to dilated cardiomyopathy or due to HOCM with diastolic dysfunction;
but mainly in diastolic heart failure and HCM we find LVF symptoms like pulmonary
edema, more right sided problems happen in dilated cardiomyopathy, the progression of
HOCM to RVF may begin at later stages, after 10-15 yrs while in dilated cardiomyopathy
it may happen after 2 yrs.

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Symptoms severity progresses with age.

Dyspnea on exertion-most common initial or presenting symptom.
Angina-like chest pain (carries the same idea as dilated cardiomyopathy), in
hypertrophied myocardium there is decrease in the percentage of the coronary
arteries that supply an area of the myocardium relative to that area
Palpitations; patients with HOCM are very likely to have arrhythmias and sudden
death, syncope may also be present due to ventricular tachycardia and it can cause
sudden death.

Fourth heart sound (S4) is a marker for
diastolic dysfunction; it is a marker of
increased end diastolic pressure.
Hyperdynamic apical impulse because of the
hypertrophied left ventricle compared to
weak apical pulse seen in dilated
Precordial lift because of the LVH.
Systolic ejection murmurs at the apex we
hear mitral regurg, and at the lower
left sternal border we hear late
systolic murmur which is a marker
for obstruction.
Murmur increased with valsalva
maneuver; when you take a deep
breath the venous return will
decrease, in case of HOCM when

Valsalva manoeuvre is performed by

forcible exhalation against a
closed airway, For example, the
Valsalva maneuver classically
increases the intensity
of hypertrophic
cardiomyopathy murmurs, viz. those
of dynamic subvalvular left
ventricular outflow obstruction;
whereas it decreases the intensity of
most other murmurs,
including aortic stenosis and atrial
septal defect

This is v. imp
Anything the contractility will the obstruction,
the pressure and it will the severity of murmurs.
Anything the preload will the obstruction, the
pressure and it will the severity of the murmurs.
anything the after-load the obstruction, the
pressure and it will the severity of the murmurs.

the venous return decrease the

obstruction will increase and the murmur will increase, thats why valsalva maneuver
decrease the obstruction, this maneuver is used to differentiate between the
murmur of HOCM and the murmur of mitral valve or aortic stenosis.

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EKG-LVH in 30%,
Left atrial enlargement In 25-50% this appears as prominent P wave in lead I and
camel hump shaped P wave in lead II
Large septal Q waves-25%
Sometimes in apical hypertrophy we will have significant T wave inversion in lead
CXR-usually normal and may show left ventricular hypertrophy and the apex is
elevated from the diaphragm.
Echo-study is the diagnostic choice-demonstrates disproportionate septal
hypertrophy of the posterior wall or the anterior wall, in some cases you may see
mitral valve regurgitation , and in cases of obstruction u may see systolic anterior
portion of the septum and mitral leaflet forming obstruction in the anterior
portion and this is characteristic for HOCM.

ED Care and Disposition

Pts who complain of exercise intolerance or chest pain with typical HOCM murmurneeds echo and referral for cardiology.
B-blocker-treatment of choice for HCM because as we said patients with
cardiomyopathies are more liable for arrhythmias and sudden death, in addition
they have a negative inotropic effect so its useful in particular in HCM.
Discourage vigorous exercise.
Patients with HCM and syncopal attacks or with HCM and HF must be admitted to
the hospital.

Restrictive Cardiomyopathy

It is the least common cardiomyopathies.

Ventricular volume and wall thickness is normal, Decreased volume of both

ventricles, the ventricular muscle is normal or hypertrophied but there is bi atrial
enlargement, so the ventricles are small and the atria are enlarged.

Mostly idiopathic and sometimes its familial

Systemic disorders associated with restrictive cardiomyopathy like amyloidosis,

sarcoidosis, hemochromatosis, scleroderma, and carcinoid.
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Size if the ventricles

Size of the atria

Type of


Both or one ventricle is


Both or one atria is




Left ventricle wall is thick

but the size is normal

Left atria is enlarged



Small ventricles with

normal muscle thickness
or hypertrophied

Large atria.

More diastolic than


Clinical Features
Symptoms of CHF and right sided HF (pulmonary edema, dyspnea, orthopnea,,PND,
lower limb edema and rarely chest pain
Exam-may have S3the marker of heart failure
or S4 gallop the marker of diastolic dysfunction,
rales, jvd, Kussmauls sign(jvd with inspiration),
hepatomegaly, pedal edema or ascites .
Shows more diastolic dysfunction rather than systolic
dysfunction, its a problem in filling the ventricles, not
emptying them.

It is a medical diagnosis

Kussmaul's sign is the observation of

a jugular venous pressure (JVP, the
filling of the jugular vein) that rises
with inspiration.
Possible causes of Kussmaul's sign

Constrictive pericarditis

Restrictive cardiomyopathy

Pericardial effusion or Cardiac


CXR-signs of CHF without cardiomegaly

EKG-nonspecific changes, most likely Conduction disturbances and low-voltage QRS
complexes, common with sarcoidosis or amyloidosis also u can see 2nd degree heart
block in amyloidosis.

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Differential Diagnosis
Constrictive pericarditis.
Diastolic left ventricular dysfunction(due to ischemic or hypertensive heart
disease) any form of diastolic dysfunction can be in the differential of restrictive
Its v imp to differentiate between restrictive cardiomyopathy from constrictive
pericarditis which needs surgical treatment while the one earlier needs treatment
of the underlying cause and treat the symptoms.
Any patient that comes to you with heart failure and normal ejection fraction and
small right and left ventricles and bi atrial enlargement it means restrictive

ED Care and Disposition

When a patient presented to the emergency room with restrictive cardiomyopathy or any
type of heart failure with dyspnea and pulmonary edema, We need to administer
diuretics, but be careful, because dieresis for a patient with diastolic dysfunction not
like dieresis in a patient with dilated cardiomyopathy and systolic dysfunction, the
rapidity of diuretic administration in patients with restrictive cardiomyopathy should be
less than that done in patients with systolic dysfunction, because in patients with HOCM
and restrictive cardiomyppathy filling of the ventricles and decreasing the obstruction
depends on the venous return, but on dieresis the venous return can decrease, and the
obstruction increases which will make the condition worsen, unlike patients with dilated
cardiomyopathy to whom dieresis is imp in high and rapid dose.
Corticosteroids for sarcoidosis
Chelation therapy for hematochromatosis
In patients with HOCM and restrictive cardiomyopathy ACEI are constructively
avoided, while we use them in dilated cardiomyopathy and heart failure.
Admission based on severity of symptoms and availability of prompt follow up.

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Dysrhythmogenic Right Ventricular Cardiomyopathy(DRVC)

It is the rarest form of cardiomyopathy, characterized by Progressive replacement of RV
myocardium with fibrofatty tissue. It is typically presented with sudden death in young
or middle aged patients.

Clinical picture and diagnosis:

Exam usually normal

EKG- RBBB may be present.

Echo-necessary for diagnosis but in most patients its normal; I shows nothing but
right ventricular dilation

The definite diagnostic test for this is cardiac MRI but we dont have it in Jordan it
shows that the right ventricle is dilated and shows abnormal texture of the right

And for 100% diagnosis, do the patient a living endocardial biopsy.


Inflammation of myocardium

Can be result of systemic

disorder like SLE and PAN (
polyarteritis nodosa) and
scleroderma or infectious

Viral 95%-Coxsackie B is the

most common virus , echovirus,
influenza, parainfluenza,
Epstein-Bar, and HIV.

Bacterial-C. Diptheria which is

rarely seen in our area, N.
meningitidis, M. pneumonia, and beta-hemolytic strep and staph the last 2 are
commonly acquired in the hospital and usually causes pneumonia but can cause
myocarditis as well.

Frequently accompanied with pericarditis

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Clinical Feature
Most of the symptoms are related the pathological features of Coxsackie B virus
Fever, tachycardia out of proportion to fever, myalgias, headache,rigors
Chest pain due to coexisting pericarditis
Pericardial friction rub
Severe cases may have CHF symptoms in the mild cases we may see left ventricular
failure symptoms

Diagnosis and Differential

EKG-nonspecific changes, av block, prolonged QRS suration, or ST elevation(with
CXR-Normal its not uncommon to see some infiltrations
Cardiac Enzymes- may be elevated
Differentail-ischemia or infarct, valvular disease, and sepsis

ED Care and Disposition

Supportive care such as bed rest and if there is low O2 saturation and lung
congestion we give oxygen therapy, give antipyretic in case of fever.
Blood cultures
Antibiotics for bacterial cause
Watch for signs of progressive heart failure

The only complication in a very small percentage of cases(510%) happens when myocarditis progress into dilated
cardiomyopathy, but most of the cases regain full recovery.

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Acute Pericarditis
Loose visceral pericardium and
dense parietal pericardium
surround heart
Pericardial space may contain up
to 50ml normally
Etiologies of acute pericarditisviral, bacterial, fungal, malignancy,
drugs, radiation, connective tissue
disorder, uremia, myxedema,
post-MI, or idiopathic

Clinical Features
Most common-sudden or gradual
onset of sharp or stabbing pain
with radiation to back, neck, L
shoulder or arm
Radiation to L trapezial ridge is
Pain more severe with lying supine and relieved with sitting
Low grade fever, dyspnea and dysphagia
Transient, intermittent friction rub

EKG-changes in four stages
1) ST elevation in I, V5 and V6, PR depression in II, aVF and V4-V6
2) ST segment normalizes, T wave decreases
3) Inverted T waves in leads with previous ST elevation
4) Return to normal ECG
In I, V5, or V6 ST:Twave ratio >0.25 most likely acute pericarditis
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Diagnosis and Differential

Chest Xray-normal and can help r/o other disease
Other tests of value-CBC, BUN and Cr, streptococcal serology, viral serologies,
antinuclear/anti-DNA abs, thyroid function, ESR, Cardiac Enzymes

ED Care and Disposition

Idiopathic or presumed viral etiology-outpatient with NSAIDs for 1-3 weeks
Treat any indentified specific causes
Admit for myocarditis or hemodynamic instability

Nontraumatic Cardiac Tamponade

Pressure in pericardial sac exceeds

normal filling pressure in RVrestricts filling and cardiac output

Etiology-metastatic malignancy,
uremia, hemorrhage(overanticoagulation), bacterial or
tubercular disorders, chronic
pericarditis, SLE, post radiation,

Clinical Features
Dyspnea and decreased exercise tolerance-wt loss, pedal edema, ascites
Tachycardia, Narrow pulse pressure
Pulsus paradoxus
JVD, Muffled heart sounds, Hypotension

Diagnosis and Differential

EKG-low voltage QRS with ST elevation and PR depression possible
Electrical Alternans-classic findingP and R wave beat to beat variability
CXR-+/- enlarged cardiac silhoutte
ECHO-diagnostic modality of choice

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ED Care and Disposition

IV Fluid Bolus-improves RV filling and improves hemodynamics
Pericardiocentesis-therapeutic and diagnostic
Admission to ICU or monitored setting

Constrictive Pericarditis
Occurs when fibrous thickening and loss
of elasticity interfere with diastolic
Cardiac trauma, pericardiotomy,

Its hard to differentiate between constrictive

pericarditis and restrictive cardiomyopathy but
we do so by:

intrapericardial hemmorhage, fungal or

bacterial pericarditis, uremic pericarditis
are most common causes

Clinical Features
Sxs gradually develop-mimics

restrictive CM- CHF, DOE, and

decreased exercise tolerance

MRI and CT scan to look for the thickening

of the pericardium, if there is thickening then
its most probably constrictive pericarditis
and we can make sure by doing x-ray because
the most common cause of constrictive
pericarditis worldwide is TB so we may see
calcification of the pericardium on the x-ray.
In some cases we cant differentiate between
them so the surgeon would do open heart
surgery and he will see by himself which it is.

Chest pain, orthopnea and pnd are uncommon

Exam-Pedal edema, hepatomegaly, ascites, jvd, and Kussmauls sign.
Pericardial knock-early diastolic sound may be heard at apex

EKG-not very helpful-may show low voltage QRS and inverted T waves
CXR-pericardial calcifications seen in 50% on lateral view(not diagnostic)
ECHO, CT, MRI are diagnostic

Differential Diagnosis
Consider acute pericarditis, myocarditis, exacerbation of chronic ventricular dysfunction,
or systemic process resulting in decreased cardiac performance (sepsis)

ED Care and Disposition

Supportive care
Symptomatic patients require admission and pericardiectomy
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Which is the 2nd most common cause of adolescent sudden death?
A. Suicide



D. Hypertrophic cardiomyopathy
Which of the following is not useful in cadiac tamponade?
A. IV fluids

B. Pericardiocentesis

Bedside ultrasound

D. Diuretics
E. Ekg
Which of the following is not found on the EKG of acute pericarditis?
A. ST elevation and PR depression
B. Normal EKG

Inverted Twave

D. Electrical Alternans
Which is not a sign or symptom of acute pericarditis?
A. Sharp stabbing chest pain with radiation to back, neck or L shoulder
B. Oslers Nodes

Friction Rub

D. Pain worse with lying supine

Which is not one of the 4 types of cardiomyopathy?
A. Infectious
B. Dilated


D. Restrictive
E. Dysrhythmic Right Ventricular

THX sawa for the recorder and happy anniversary

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