Cardiomyopathy is a disease of the heart muscle that can cause mechanical and electrical dysfunction. It has many causes including genetic disorders, infections, and metabolic issues. The prognosis for cardiomyopathy is generally poor if left undiagnosed until advanced stages. Diagnostic tools include echocardiography, chest x-rays, and cardiac catheterization. Dilated cardiomyopathy is the most common type and is characterized by the enlargement of heart chambers and thinning of the walls. It can lead to heart failure if not properly managed with medications like ACE inhibitors, beta blockers, diuretics, and devices like defibrillators if needed. The prognosis is poor with 50% of patients dying within

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Cardiomyopathies
 ` 2006 AHA defined cardiomyopathies as “a heterogeneous group of
diseases of the myocardium associated with mechanical &/or electrical
dysfunction that usually (but not invariably) exhibit inappropriate
ventricular hypertrophy or dilatation and are due to a variety of causes
that frequently are genetic.”
FACTS: Cardiomyopathy is the 2nd
most common cause of sudden death
Prognosis for Cardiomyopathy is very poor
** Undiagnosed until in advanced stages **
DIAGNOSTIC EVALUATION:
Echocardiography → confirms dilated
cardiomyopathy
Chest X-Ray → reveals cardiomegaly associated
with any of the cardiomyopathies
Cardiac Cath with possible Biopsy → can be
definitive in diagnosing hypertrophic
cardiomyopathy
Most cardiac disease is secondary to some other condition (e.g., coronary
atherosclerosis, hypertension, or valvular heart disease). However, there are
some that are attributable to intrinsic myocardial dysfunction. Such myocardial
diseases are termed cardiomyopathies ( heart muscle diseases).

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
They are a diverse group that includes inflammatory disorders (myocarditis),
immunologic diseases (e.g., sarcoidosis), systemic metabolic disorders (e.g.,
hemochromatosis), muscular dystrophies, and genetic disorders of cardiac
muscle cells. In many cases, cardiomyopathies are of unknown etiology (termed
idiopathic); however, several previously "idiopathic" cardiomyopathies have
been shown to be caused by specific genetic abnormalities in cardiac energy
metabolism or structural and contractile proteins.
Classification

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Dilated Cardiomyopathy(DCM)
 DCM is most common of all CMs(60%)
 Dilated cardiomyopathy (DCM) is characterized by progressive cardiac
dilation and contractile (systolic) dysfunction. It is sometimes called
congestive cardiomyopathy. Approximately 25% to 50% of DCM cases
have a familial (genetic) basis. Others result from a variety of acquired
myocardial insults including toxic exposures (e.g., chronic alcoholism),
myocarditis, and pregnancy . In some patients, the cause of DCM is
unknown. Such cases are appropriately called idiopathic dilated
cardiomyopathy. Many in this category are likely to be of genetic origin.
Regardless of the cause, all share a similar clinicopathologic picture.
 Aetiology
Idiopathic (IDC)30%
Myocarditis (9%) Viral / Bacterial Infection
Ischemic (7%)
Genetic disorders 50%(AD)
Hypertension
Hyperthyroidism
Valvular Heart Disease
Chemotherapy
Peripartum CMP
Cardiotoxic Effects of Drugs or alcohol
 Morphologically
 The heart in DCM is characteristically enlarged (two to three times its
normal weight) and flabby, with dilation of all chambers.
 Because of the wall thinning that accompanies dilation, the ventricular
thickness may be less than, equal to normal. Mural thrombi are common
and may be a source of thromboemboli.

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 By definition there is no primary valve pathology; consequently, any
valvular insufficiency is a secondary consequence of ventricular chamber
dilation. The coronary arteries are usually free of atherosclerotic stenosis.
Enlargement of RV & LV cavities without an increase in ventricular septal
or free wall thickness → spherical shape & dilatation of heart →
Displacement of papillary muscles → Regurgitant lesions despite valve
leaflets being normal
Pathophysiology:
 Microscopically – fibrosis & scarring
 Systolic Dysfunction>>> Diastolic dysfunction
 STROKE VOLUME is initially maintained by ↑↑ EDV
 With disease progression→Marked LV dilatation with normal or thin wall
→↑ Wall stress + Valvular Regurgitation →Overt Circulatory Failure .

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Clinical Features
 Symptoms: GRADUAL , MIDDLE AGE, MIMIKING URTI
The fundamental defect in DCM is ineffective contraction. Hence in end-
stage DCM, the cardiac ejection fraction is typically less than 25%.
Typically pts c/o months of fatigue, weakness, reduced exercise tolerance
Symptomatic HF (syncope, dyspnea, volume overload)
LVF- Dyspnea, PND, orthopnea, cough, frothy sputum, palpitation
RVF-Odema, abdominal swelling,constipation, RT hypochondrial pain
Some patients are asymptomatic
May also present as a Stroke, Arrythmia or Sudden Death.
Fifty percent of patients die within 2 years, and only 25% survive longer
than 5 years
 Physical Signs( signs of heart failure)
Tachycardia , pulsus alternans
Jugular venous distension
Pulse pressure is narrow
Variable degrees of cardiac enlargement
Displace apical impulse
3rd
or 4th
heart sound are common (with the bell)
MR=blowing scratchy systolic
Mitral/ tricuspid regurgitation may occur
lung--Diminished breath sounds with effusions
Rales if in failure
Summary==== symptoms and signs of heart failure
Diagnosis:
 CXR- Cardiomegaly , Pulmonary venous congestion ,pleural effusion
 ECG- Normal or low QRS voltage , abnormal axis, non specific ST seg
abnormalities, LV hypertrophy, conduction defects, Non sustained
Ventricular tachycardia/Sinus tachycardia/atrial fibrillation, Left atrial
abnormality, LBBB

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 2D Echo
Chamber size - Wall thickness/shape( Eccentric hypertrophy/ Usually thin)
Clot formation
Ejection Fraction -Normal 55-65%//Mild Dysfunction 41-55%
Moderate Dysfunction -----------26-40%
Severe Dysfunction----------------<26%
 Coronary Angiography- age >40
-usually normal coronaries
-coronary vasodilatation is impaired by ↑ LV filling pressures
-distinguishes b/w Ischemic & Idiopathic DCM
 Endomyocardial Biopsy
rarely valuable to identify the aetiology

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Management
 Aim of treatment
-Manage the symptoms
-Reduce the progression of disease
-Prevent Complications
 salt restriction of a 2-g Na+
(5g NaCl) diet
 fluid restriction for significant low Na+
 Mainstay of Therapy
Vasodilators + Digoxin + Diuretics + BB(CARDIOSELECTIVE)
Reduce preload Reduce afterload
_ Diuretics Arterial Vasodilators
_ Venous Vasodilators ACE Inhibitors
_ ACE Inhibitors
_ Aldosterone antagonists
Increase Contractility
_ Decrease afterload
_ Digoxin
Vasodilators (afterload reducing drugs)
 ACE Inhibitors
-Indicated for all patients
- Reduce symptoms & improve effort tolerance
- Suppress ventricular remodelling & endothelial dysfunction
-Reduce CV mortality
 Spironolactone
Used along with ACE Inhibitors has shown to reduce mortality by 30% in a
large double blind randomized trial
 Digoxin
clinically beneficial as reaffirmed by two large trials in adults

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 β Blockers- carvedilol
They provide symptomatic improvement and substantial reduction in
sudden death in NYHA class II & III HF pts
 Amiodarone
-High grade ventricular arrhythmias (Sustained VT or VF) are common in
DCM→↑ risk of SCD
-Preferred anti arrhythmic agent as it has least negative inotropic effect
& proarrhythmogenic potential
-Implantable Defibrillators are used for refractory arrhythmias.
 Anticoagulants
-Indicated for pts with moderate ventricular dilatation+mod-severe
systolic dysfunction
-H/O stroke , AF or evidence of Intracardiac thrombus
 Hydralazine / nitrate combination
 anticoagulation for EF <30%, history of thromboemoli, presence of mural
thrombi
 intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors
Pts refractory to Pharmacological therapy for CHF
 Dual Chamber Pacing
 Cardiomyoplasty
 LV Assist Devices
improved pts sufficiently to avoid transplant or enable later transplant
 Cardiac Transplantation
has substantially prolonged survival in DCM pts with 5 yr survival rate of
78%.

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM) (also known as idiopathic hypertrophic
subaortic stenosis) is characterized by myocardial hypertrophy, abnormal
diastolic filling, and-in a third of cases-ventricular outflow obstruction. The
obstruction, in some cases, is dynamic, caused by the anterior leaflet of the
mitral valve. The heart is thick-walled, heavy, and hyper contracting. Systolic
function is usually preserved in HCM, but the myocardium does not relax and
therefore shows primary diastolic dysfunction.
 Most common cause of sudden death in young athletes.
 Characterized by in appropriate and elaborate LV hypertrophy with
misalignment of the myocardial fibres.
 Hypertrophy may be generalized or confined largely to interventricular
septum
Heart failure may develop because stiff non-compliant ventricles impede
systolic filling and decreased cardiac output
Septal hypertrophy may cause dynamic LV outflow obstruction.
Mitral regurgitation occur due to abnormal systolic anterior mitral valve
leaflet.
REMMEBER:
Primary genetic cardiomyopathy and left ventricle disease.
Effects men and women equally
Hypertrophy of myocardial muscle mass
Cause - transmitted genetically(autosomal dominant)
Disarray of cardiac myofibrils with hypertrophy of myocytes
Cells take on a variety of shapes
Myocardial scarring and fibrosis occurs

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Pathophysiology
 Condition is genetic disorder with Autosomal Dominant transmission.
 Due to single point mutation in one of the genes that encode sarcomeric
contractile proteins.
 Morphology :
 The essential gross feature of HCM is massive myocardial hypertrophy
without ventricular
dilation .The classic
pattern of HCM involves
disproportionate
thickening of the
ventricular septum
relative to the left
ventricle free wall (so-
called asymmetrical
septal hypertrophy);
nevertheless, in about
10% of cases there is
concentric hypertrophy.
On longitudinal
sectioning, the
ventricular cavity loses
its usual round-to-ovoid
shape and is compressed
into a "banana-like"
configuration .Often present is an endocardial plaque in the left
ventricular outflow tract, as well as a thickening of the anterior mitral
leaflet. Both findings reflect contact of the anterior mitral leaflet with the
septum during ventricular systole and correlate with functional left
ventricular outflow tract obstruction.

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Pathophysiology( post graduate)
1. Subaortic Obstruction
2. Diastolic Dysfunction
3. Myocardial Ischemia
4. Mitral Regurgitation
5. Arrythmias
A. Subaortic Obstruction
1. Cause -Assymetrical Septal Myocardial Hypertrophy .
2. Subaortic Obstruction
Effect-Systolic anterior motion(SAM) of AML → accentuating obstruction
Mechanism of SAM
Thickened IVS→Restricted LVOT → ejection of blood at a higher velocity
closer to the AML → Drawing of AML closure towards the hypertrophied
septum due to the venturi effect → Dynamic LVOT obstruction
Factors aggregating SAM and cause dynamic obstruction(30-50%):
↑ Contractility
↓ Afterload (Aortic outflow resistance)
↓ Preload (End diastolic volume)
 Therapeutically Myocardial depression, Vasoconstriction & Volume
overloading should minimize obstruction & augment forward flow
B. Diastolic Dysfunction
STIFF LEFT VENTRICLE/IMPAIRED RELAXATION/PASSIVE FILLING
ATRIAL KICK (S4) IS ESSENTIAL TO MAINTAIN CARDIAC OUT PUT
DECREASE LV CAVITY= DIASTOLIC DYSFUNCTION= LV FILLING

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
FILLING PRESSURES & PULMONARY CONGESTION
Compensation for decreased filling -> hyperdynamic systolic
Dysfunction ------------EF increases to 70-80%
c.Mitral Regurgitation
– Hypertrophied papillary muscles
– Leaflets become calcified and thick
– Atrial dilatation
_ Enlargement of mitral valve annulus
D. Myocardial Ischemia
◦ Often occurs with normal coronary arteries.
◦ Postulated mechanisms
 Abnormally small coronary arteries as a result of hypertrophy
 Inadequate number of capillaries for the degree of LV mass
 Early closure of aortic valve with decreased cardiac output
 Increased myocardial oxygen demand due to :
1. Myocardial hypertrophy
2. Diastolic dysfunction
3. Myocyte disarray
4. LVOT obstruction
5. Arrhythmia
Types Hypertrophic Cardiomyopathy
• Asymmetric septal (ASH) - without obstruction
• Asymmetric septal (ASH) - with obstruction
• Symmetric hypertrophy - concentric
• Apical hypertrophy
• HCM or HOCM

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Signs & Symptoms
HCM is characterized by a massively hypertrophied left ventricle with diastolic
failure and systolic dysfunction. In addition, roughly 25% of patients have
dynamic obstruction to the left ventricular outflow by the anterior leaflet of the
mitral valve.
 Many asymptomatic for years
 Incidence of sudden death often first presentation
 Identified during screening of relative of patient with HCM
 Symptoms related to severity of diastolic dysfunction or
mitral regurgitation.
1. Dyspnea on exertion (90%)/activity intolerance-LVF , HF ,ISCHEMIA
2. Angina on effort (70-80%)
3. Syncope on effort (20%)-LVOT
4. Palpitations
5. Sudden cardiac death
1. Ventricular wall mass > 30 mm increased risk
2. More common in patients under 40
3. Normally occurs during strenuous activity
SIGNS(( MR+ AS))
 Pulse- Irregular Pulse (with Atrial-fibrillation)
 Bisferiens Carotid Pulse (HOCM)
– Brisk initial upstroke/ jerky pulse
– Collapse of pulse then secondary rise
– Must differentiate from AS – delayed upstroke
 Apex beat- forceful and brisk/LV heave /Double impulse at apex
 S4
 MR murmur-Pansystolic murmur
Systolic murmur with obstructive disease process

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Sign of LV outflow obstruction- Mid systolic, best heard along left sternal
boarder, usually does not radiate, crescendo-decrescendo, harsh or rough
HOCM murmur louder during Valsalva’s maneuver
 Decreases venous return to the heart
– Decreased preload _ _ Decreased left ventricular filling _ _obstruction
 Any factor that decreases venous return to the heart increases the
murmur in HOCM
– Squatting increases venous return
– Standing decreases venous return
 Aortic stenosis murmur becomes quieter during Valsalva’s maneuver
Diagnosis:
.2/3 of patients have family H/O, the rest have sporadic mutations (de novo)
 HIGH INDEX OF SUSPECION IN YOUNG MALE WITH SYNCOPY.
ECG-It is abnormal in 75 to 95% of patients, ↑ QRS voltage, ST-T changes, Axis
deviation, LV Hypertrophy +strain pattern, p wave abnormalities, Negative T
Waves in V3-V5 (apical HCM),Abnormal Q waves may reflect septal hypertrophy
CXR-Lt atrial enlargement or normal
Echo Wall thickness -LV size, Hyperdynamic LV function, Atrial size
MV leaflets, LV outflow obstruction

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Management
Goals
– Relief of symptoms- Relax the ventricle
Slow the Heart Rate
– Use Negative Inotropes
– Preventing complications
– Preventing or reducing risk of sudden death
– No evidence to support treatment of nonsymptomatic patients
DRUGS
 B-blocker and rate-limiting calcium antagonists (eg verapamil)
 Most patients are improved by therapy that promotes ventricular
relaxation.
 β Blockers- mainstay of therapy
Relieves symptoms of exercise intolerance & dyspnoea associated with

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
CHF by- negative inotropic effect
-HR reduction , lower myocardial O2 demand
- longer diastolic filling times , improve filling of LV
 CCB-Verapamil is indicated if β Blockers not tolerated or ineffective
-It improves diastolic function & ventricular relaxation causing improved
filling, decreased obstructive features in 50% pts
-CCBs with strong vasodilatory effect are C/I in pts with obstructive
symptoms
 Cardioversion (ATRIAL-Fib to Sinus)
 Anticoagulants
 Amidarone
 ACE-I AND NGT to be avoided.
 Diuretic - with caution
SURGERY:
Indications
1. Subaortic gradients≥ 50mmHg frequently associated with CHF & are
refractory to medication
2. Marked outflow obstruction
3. On maximum medical therapy
4. NYHA Class III or IV
 Septal Myotomy +Partial Mymectomy through a transaortic approach
relieves the obstruction, reduces the LVOTO gradient, SAM & MR
 MV Replacement or repair at same time (increases operative mortality)
 Improvement noted immediately and last 20-30 years
 May need pacer due to development of LBBB.
 Complications –CHB or septal perforation (0-2%)
 Mortality rate-1to 3%
 Percutaneous Alcohol SeptalAblation
Not appropriate if MVR needed, Better for patients > 55
Catheter in septal perforator
Ethyl alcohol injected -MI occurs-Enlarged septum eventually shrinks

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Ablation of AV Node (HOCM),Dual Chamber Pacemaker (HOCM)
Heart Transplant
COMPLICATION:
1. Atrial fibrillation, VT , V.fibrillation.
2. Mural thrombus formation
3. IE of the mitral valve
4. CHF
5. Sudden death. Prognosis
Adults - 2-3% SCD per y
Adolescents - 4-6% SCD per year
MARKERS OF INCREASED RISK
1. Prior cardiac arrest or spontaneous sustained VT.
2. Family history of premature HCM death
3. Syncope or near syncope (exertional or recurrent)
4. Multiple and repetitive burst of NSVT
5. Hypotensive response of BP to exercise
6. LVH greater than 30 mm(septum)
7. Myocardial ischemia (with angina)

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Restrictive CM
WHO in 1995 defined RCM as restrictive filling & reduced diastolic volume of
either or both ventricles with normal or near normal systolic function & wall
thickness.
Restrictive cardiomyopathy is characterized by a primary decrease in ventricular
compliance, resulting in impaired ventricular filling during diastole ( the wall is
stiffer). The contractile (systolic) function of the left ventricle is usually
unaffected. Thus, the
functional state can be
confused with that of
constrictive
pericarditis or
hypertrophic
cardiomyopathy.
Restrictive
cardiomyopathy can
be idiopathic or
associated with
systemic diseases that
also happen to affect
the myocardium .
Least common form of
Cardiomyopathy
5% of 1ry heart muscle Disease.
 Ventricular filling is impaired because ventricles are stiff.
 Lead to high atrial pressure with atrial hypertrophy, dilatation and later
atrial fibrillation.
 Amyloidosis is the most common cause

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Pathophysiology:
Ventricular chamber has limited ability to expand during filling (diastolic
dysfunction)- Filling Impairment- Rate of LV filling is slow
Decreased volume available to eject- Elevated LV filling pressure
Pressure Overload -------- Often elevated LV EF.
stroke volume & cardiac output
1. volume and pressure in atria(INCREASE)
2.Dilated atrium due to increase volume & pressure
3.Increase volume & pressure in pulmonary system
4.Heart failure symptoms
3 problems:
1. Stiffness of the ventricle
2. Ventricular Filling Reduced
3. The rigidity of the myocardium causes failure to completely contract
during systole
-------------((End-result is decreased CO))----------
CAUSES
 Primary---idiopathic
• MYOCARDIAL
1. Noninfiltrative
Idiopathic, Scleroderma
2. Infiltrative
Amyloid 90% north AMERICA ,Sarcoidosis ,Gaucher disease,
Radiation carditis , Hurler disease
3. Storage Disease
Hemochromatosis, Fabry disease, Glycogen storage
• ENDOMYOCARDIAL
Endomyocardial fibrosis, Hyperesinophilic syndrome, Carcinoid
metastatic malignancies, radiation, anthracycline
Signs & Symptoms:
HEART FAILURE IS THE END RESULT OF CARDIOMYOPATHY.

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Fatigue, weakness ,Syncope
Palpitations with arrhythmias
Dyspnea, Orthopnea
Edema
Chest Pain
SIGNS –
Pale/ cool-------Peripheral pulse decreased, Pulsus paradoxus
JVP-prominent x and y descents Kussmaul’s sign-↑ JVP during inspiration
Murmur of Mitral Regurgitation-----Systolic Murmur, 5th ICS MCL
M.R.- Dilation of atrium , Papillary muscle dysfunction, Fibrosis of leaflets
S4----------Left Lateral Position, Bell of Stethoscope
Diagnosis:
 Diagnosis is difficult
 Rule Out Other Causes of Diastolic Dysfunction
a. Aortic Stenosis
b. Hypertrophic Cardiomyopathy
c. Hypertensive Cardiovascular Disease
 Differentiate from Constrictive Pericarditis.
Clinical Features
Constrictive
Pericarditis
Restrictive
Cardiomyopathy
History
Prior history of
pericarditis or
condition that causes
pericardial disease
History of systemic
disease (e.g..
Amyloidosis,
Hemochromatosis)
Heart Sounds
Pericardial knock, high
frequency sound
Presence of loud
diastolic filling
sound S3, low frequency
sound
Murmurs No murmurs
Murmurs of mitral and
tricuspid
insufficiency
Arrhythmias

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Heart Pressures
L & R filling pressures up
and
equal
(Elevated JVP)
L sided filling pressures >
R sided
filling pressures
 Require complex Doppler echocardiography
CXR- pulmonary congestion, small heart size, Dilated atrium ,Congestion if in HF
Calcified pericardium can be seen inconstrictive pericarditis.
 ECG- Low QRS voltage, No-specific ST-T wave Changes, P wave
abnormalities,Arrhythmias,Conduction abnormalities, BBBs, low voltage,
QR or QS complexes, An abnormal heart rhythm is customary
• Echo-Doppler
 abnormal mitral inflow pattern
 prominent E wave (rapid diastolic filling)
 reduced deceleration time ( LA pressure)
Impaired ventricular relaxation & abnormal Compliance causes rapid
filling in early diastole & impeded filling during rest of diastole
Characteristic
Ventricular diastolic waveform of Dip & Plateau (Square root sign)
-RA pressure waveform-M or W shaped due to rapid y descent

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 Pressure in the ventricle rises precipitously in response to small volume
 Both ventricles appear thick(Increased in infiltrative disorders )with small
cavities in contrast to corresponding dilated atria
 Lt sided Pulmonary venous pressure >Rt sided venous pressure by 5mmHg
 PASP↑↑ upto 50mmHg
 Speckled appearance on myocardium with amyloidosis
Cardiac Catheratization
 Full cath not necessary -Hemodynamic measurements valuable
_ Elevated LVEDP
_ Elevated PAOP
_ Elevated RA Pressures
_ Elevated pulmonary pressures
 CT and MRI and endomyocardial biopsy may be done.
Endomyocardial Biopsy
Septal wall of RV, Multiple sites
Essential for diagnosis of RCM
Exclusion “Guidelines
• LV end-diastolic dimensions  7 cm
• Myocardial wall thickness  1.7 cm
• LV end-diastolic volume  150 mL/m2
• LV ejection fraction < 20%

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
TREATMENT
There is no cure for the disease. Similar to most types of cardiomyopathies
there are ways to reduce symptoms and prevention.
The purpose OF medical treatment is to alleviate the problems of heart rate
and prevent blood clots.
Transplantation is the best treatment.
 Idiopathic
Diuretics-To relieve congestion
B-blockers, Amiodarne, CCBs- Control of HR
vasodilators may decrease filling pressure
Long term anticoagulation-MR,TR,LA LARGE,AF, FIBROSIS
CCBs, ACEI- To enhance myocardial relaxation
Dual Chamber Pacing- AV block
Cardiac Transplantation- Refractory Heart Failure
 Amyloidosis- Melphelan, prednisone, H+L transplant
 Haemochromatosis- Phlebotomy, Desferrioxamine
 Treat Rhythm
 AF Control - Loss of atrial kick, Digoxin cautiously in amyloidosis
 Conduction abnormalities ---------May require pacemaker
Outcomes
_ Poorest mortality of all cardiomyopathies
_ 90% mortality rate at 10 years (Kavinsky & Parrillo, 2000).
_ Amyloid Heart 80% mortality at 2 years
Arrhythmogenic Right Ventricular Dysplasia
 Patches of the right ventricular myocardium are replaced with fibrous
and fatty tissue.
 Inherited as autosomal dominant trait
 Dominant clinical problems are
 ventricular arrhythmia
 Sudden death
 Right sided cardiac failure
 ECG shows inverted T waves in the right precordial leads.
 MRI useful diagnostic tool and used to screen 1st
degree relatives from
having the same pathology.

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Obliterative Cardiomyopathy
 Involves the endocardium of one or both ventricles and is characterized
by thrombosis and elaborate fibrosis with gradual obliteration of the
ventricular cavities.
 Mitral and tricuspid valves are regurgitant.
 Heart failure and pulmonary and systemic embolism are prominent.
 Associated with eosinophilia
 Eg: eosinophilic leukemia, churg strauss syndrome
 Mortality is high (50% at 2 years of developing the symptoms)
 Anticoagulation and antiplatelet therapy is advisable and diuretics may
help symptoms of HF.
 Surgery valve replacement with decortication of endocardium may be
helpful in certain cases.
Myocarditis
In myocarditis there is inflammation of the myocardium with resulting injury.
It is important, however, to emphasize that the presence of inflammation
alone is not diagnostic of myocarditis; for example, inflammatory infiltrates
can also occur as a secondary response to ischemic injury. In myocarditis, the
inflammatory process is the cause of-rather than a response to-myocardial
injury.
Lymphocytic myocarditis is most common .If the patient survives the acute
phase of myocarditis, the inflammatory lesions either resolve, leaving no
residual changes, or heal by progressive fibrosis
Pathogenesis
INFECTIONS Viruses-coxsackie virus, HIV, influenza,ECHO,cytomealovirus
Chlamydiae Psittaci
Rickettsiae typhi , typhus fever
Bacteria-Diphtheriae, Neisseria meningeocccus,Lyme disease
Fungus- Candida
Protozoa-Trypanosome (chagas disease), Toxoplasmosis
Helminths- Trichinosis

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
IMMUNE MEDIATED REACTIONS
Post viral
Post streptococcal-Rheumatic fever
Systemic lupus erythematosus
Transplant rejection
Drug hypersensitivity- methyl dopa, sulphonamides
UNKNOWN
Giant cell myocarditis
Sarcoidosis
Clinical Features
The clinical spectrum of myocarditis is broad.
At one end, the disease is asymptomatic and patients recover without
sequelae, and at the other end is the precipitous onset of heart failure or
arrhythmias, occasionally with sudden death.
Between these extremes are the many forms of presentation, associated with a
variety of symptoms (e.g., fatigue, dyspnea, palpitations, pain, and fever).
The clinical features of myocarditis can even mimic those of acute MI.
Occasionally, over many years, patients can progress from myocarditis to DCM
Clinical Presentation
Acute Fulminant Chronic
 Nonspecific cardiac
symptoms
 Heart failure, Acute
MI, or SCD
 More common in
children/teenagers
 +/- viral prodrome
 Cardiogenic shock
+/- acute heart failure
 Biopsy doesn’t match
the clinical severity.
 High levels of
cytokines 
reversible cardiac
depression  better
prognosis
 Subtle, insidious
onset
 Already have DCM
HF symptoms
 Biopsy with fibrosis
usually

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Diagnosis:
Symptoms: non-specific
Laboratory Testing: also non-specific
 Positive cardiac biomarkers
 ECG: T wave inversion, ST segment elevation, bundle branch blocks
ECHO
 Differentiate fulminant from acute myocarditis
 Detect thrombi, valvular abnormalities, and pericardial involvement
 Rule out other cardiomyopathies (HOCM, Takotsubo)
Cardiac MRI
Non-invasive
Visualize entire
myocardium
Use to guide biopsy
Follow disease course
and response to therapy
Coronary Angiography
 Rule out other congenital, rheumatic, or ischemic heart disease
 Determine need for inotropic or mechanical support based on
hemodynamic parameters
 Elevated pulmonary artery pressures are independent predictors of
mortality
Endomyocardial Biopsy
 Although controversial, still the current gold-standard test for diagnosis
 1-6% complication rate
 Consider when suspicious for:

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
1. Giant cell myocarditis
2. Hypersensitivity/eosinophilic myocarditis
3. Cardiac involvement in a systemic disease
 All other patients, consider only if pt is deteriorating
Treatment
 Circulation:
 Intra-aortic balloon pump counterpulsation
 Ventricular assist device
 Cardiopulmonary assist device
 Medical therapy
1. ACE-inhibitors
2. Beta-blockers
Most therapy used in HF patients appears to benefit those with HF due to
myocarditis – with the exception of digoxin
 Immunosuppressive therapy- N0 RULE

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 Intra-aortic balloon pump
 Electrocardiographic synchronized phased pulsation
o Inflation with aortic valve closure
o Deflation just before systole
 Reduce systolic arterial pressure (afterload)
o Reduces myocardial oxygen consumption
 Augment diastolic arterial pressure
o Enhances coronary blood flow
 Mean pressure unchanged
Intra-aortic balloon pump
 Benefits:
 Diminish myocardial
ischemia
 10-20% increase in CO
 Diminish heart rate
 Increase urine output
 Risks:
 Damage/perforation of
aorta
 Distal ischemia
 Thrombocytopenia
 Hemolysis
 Renal emboli
 Mechanical failure –
balloon rupture
Ventricular-assist device
 Centrifugal pump or Archimedes’ screw type
 Inflow from LV and outflow into aorta
 Has been used as a bridge in myocarditis until recovery or transplant

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
 Disadvantages:
1. Surgical implantation
2. infection
3. thrombosis
4. hemolysis

*
*Centrifugal pump vs. corkscrew
“Loose” rule of third’s…
 1/3: recover
 1/3: residual ventricular dysfunction
 1/3: transplantation or death

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MAGDI AWAD SASI CARDIOMYOPATHY 2013
Diagnosis
 Expanded Criteria
 Suspicious for
myocarditis = 2 positive
categories
 Compatible with
myocarditis = 3 positive
categories
 High probability of
being myocarditis = all 4
categories positive
 Category I: Clinical symptoms
 Category II: Evidence of Cardiac
dysfunction in the Absence of
regional coronary ischemia
 Category III: Cardiac MRI
 Category IV: Myocardial biopsy
- Pathological or Molecular
Analysis
 Most common cause is viruses (adeno and coxsackie)
 Highly variable clinical manifestations
 Cardiac MRI looks promising for diagnosis
 Biopsy is the gold standard but should be pursued in only select patients
 Aggressive, supportive care is the first line therapy because of high
incidence of recovery
 Immunosuppressive therapy does not affect mortality
 Supportive therapy is mainstay therapy
 Most medical therapies for HF seem to benefit myocarditis patients with
the exception of digoxin

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 Immunosuppressive therapy does not seem to play a role in survival
BIOPSY