CARDIOMYOPATHY

1
Definition
Disorders of the cardiac muscle of unknown
aetiology
A primary disorder of the heart muscle that
causes abnormal myocardial performance and is
not the result of disease or dysfunction of other
cardiac structures myocardial infarction,
systemic hypertension, valvular stenosis or
regurgitation



2
Classifications
Braunwald 8th ed, 2008.
3
Functional Classification
Dilatated (congestive, DCM, IDC)
ventricular enlargement and syst dysfunction
Hypertrophic (IHSS, HCM, HOCM)
inappropriate myocardial hypertrophy
in the absence of HTN or aortic stenosis
Restrictive (infiltrative)
abnormal filling and diastolic function

4
IDIOPATHIC DILATED CARDIOMYPATHY
EPIDEMIOLOGY
ANNUAL INCIDENCE 5-8/100,000

PREVELANCE 36/ 100,000

INCREASED RISK ASSOCIATED WITH:
MALE GENDER
BLACK RACE
HYPERTENSION
CHRONIC BETA-AGONIST USE
5
ETIOLOGIES OF DILATED CARDIOMYOPATHY
0
5
10
15
20
25
30
35
40
45
50
Disorder
IDCM
Myocarditis
Ischmic CM
Infiltrative
disease
Peripartum CM
Hypertension
HIV
CTD
Substance
abuse
Felker et al NEJM 2000
6
IDIOPATHIC DILATED CARDIOMYOPATHY
PATHOLOGY
Four chamber dilatation
Mild to moderate ventricular
hypertrophy
Varying degrees of interstitial
fibrosis and myocyte
hypertrophy
Functional atrioventricular
regurgitation is common
Normal epicardial coronary
arteries

7
IDIOPATHIC DILATED CARDIOMYOPATHY
PATHOGENESIS
Familial/genetic factors
Viral myocarditis and cytotoxic insults
Immunologic abnormalities
Beta-receptor auto-antibodies
Abnormal T-cell function
Metabolic, energetic, and contractile
abnormalities
Ca
2+
-ATPase
Myofibrillar ATPase
Creatine Kinase

8
MOLECULAR DEFECTS IN DILATED
CARDIOMYOPATHY
Fatkin D, et al. NEJM 1999;341
GENES
Lamin A/C
-sarcoglycan
Dystrophin
Desmin
Vinculin
Titin
Troponin-T
-tropomyosin
-myosin heavy chain
Actin
Mitochondrial DNA
mutations
9
IDIOPATHIC DILATED CARDIOMYPATHY
CLINICAL PRESENTATIONS
Heart failure symptoms 75%-85%
Anginal chest pain 8%-20%
Emboli (systemic or pulmonary) 1%-4%
Syncope <1%
Sudden cardiac death <1%
10
IDIOPATHIC DILATED CARDIOMYPATHY
CARDIAC IMAGING
CXR : enlargement of cardiac silhoutte
ECG : evidence of old MCI, conduction abnormalities
e.g LBBB, LV hypertrophy, AF or VT
24-hour ambulatory ECG (Holter)
lightheadedness, palpitation, syncope
Two-dimensional echocardiogram or Radionuclide
ventriculography to assess : LV ejection fraction, end-
diastolic volume, diastolic function
Cardiac catheterization
age >40, ischemic history, high risk profile, abnormal ECG
11
IDCM:PROGNOSTIC FEATURES
VENTRICULOGRAPHIC FINDINGS
Degree of impairment in LVEF
Extent of left ventricular enlargement
Coexistent right ventricular dysfunction
Ventricular mass/volume ratio
Global wall motion abnormalities
Left ventricular sphericity

CLINICAL FINDINGS
Favorable prognosis: NYHA < IV, younger age, female
sex

Poor prognosis: Syncope, persistent S3 gallop, right-
sided heart failure, AV or bundle branch block,
hyponatremia, troponin elevation, increased BNP,
maximum oxygen uptake < 12 mg/kg/min
12
IDIOPATHIC DILATED CARDIOMYPATHY
PREDICTING PROGNOSIS
Predictive Possible Not Predictive
Clinical factors symptoms alcoholism age
peripartum duration
family history viral illness

Hemodynamics LVEF LV size
Cardiac index atrial pressure

Dysarrhythmia LV cond delay AV block simple VPC
complex VPC atrial fibrillation

Histology myofibril volume other findings

Neuroendocrine hyponatremia
plasma norepinephrine
atrial natriuretic factor
13
RIGHT
VENTRICULAR
BIOPSY
TECHNIQUE
ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY
14
INDICATIONS FOR ENDOMYOCARDIAL
BIOPSY
Acute dilated cardiomyopathy with refractory heart failure
symptoms
Rapidly progressive ventricular dysfunction in an
unexplained cardiomyopathy of recent onset
New onset cardiomyopathy with recurrent ventricular
tachycardia or high grade heart block
Heart failure in the setting of fever, rash, and peripheral
eosinophilia
Dilated cardiomyopathy in setting of systemic diseases
known to affect the myocardium (systemic lupus erythematosus,
polymyositis, sarcoidosis)

Wu LA, et al. Mayo Clin Proc 2001;76:1030-8
15
IDIOPATHIC DILATED CARDIOMYOPATHY
MANAGEMENT
Limit activity based on functional status
salt restriction of a 2-g Na
+
(5g NaCl) diet
fluid restriction for significant low Na+
initiate medical therapy
ACE inhibitors, diuretics
digoxin, carvedilol
hydralazine / nitrate combination
16
IDIOPATHIC DILATED CARDIOMYOPATHY
MANAGEMENT
consider adding -blocking agents if
symptoms persists
anticoagulation for EF <30%, history of
thromboemoli, presence of mural thrombi
intravenous dopamine, dobutamine and/or
phosphodiesterase inhibitors
cardiac transplantation
17
HYPERTROPHIC CARDIOMYOPATHY

First described by the French and Germans
around 1900
uncommon with occurrence of 0.02 to 0.2%
a hypertrophied and non-dilated left ventricle in
the absence of another disease
small LV cavity, asymmetrical septal
hypertrophy (ASH), systolic anterior motion
of the mitral valve leaflet (SAM)
18
HYPERTROPHIC CARDIOMYOPATHY
EPIDEMIOLOGY
Prevalence : 1 per 500 in
US
Higher in Black individual
Inherited in an
autosomal dominant
pattern
Predominance in
younger Male
Commonest cause of
sudden death during
exertion in young people
19
HYPERTROPHIC CARDIOMYOPATHY
PATHOGENESIS
Misconception that outflow tract obstruction
Impaired ventricular compliance as
consequence of inappropriate myocardial
hypertrophy
20
HYPERTROPHIC CARDIOMYOPATHY
PATOPHYSIOLOGY
Systole
dynamic outflow tract gradient
Diastole
impaired diastolic filling, filling pressure
Myocardial ischemia
muscle mass, filling pressure, O2 demand
vasodilator reserve, capillary density
abnormal intramural coronary arteries
systolic compression of arteries
21
HYPERTROPHIC CARDIOMYOPATHY
MYOCARDIAL DISARRAY
Normal Muscle Structure Myocardial Disarray
(Parallel alignment) (Disorganised alignment)
22
HYPERTROPHIC CARDIOMYOPATHY
TYPES

HCM or HOCM
Asymmetric septal (ASH) - without obstruction
Asymmetric septal (ASH) - with obstruction
Symmetric hypertrophy - concentric
Apical hypertrophy
23
65% 35%
10%
www.kanter.com/hcm
24
HCM
Mitral
Valve in
normal
position
25
HOCM
Mitral valve
presses
against
septum
MR
26
SYMMETRIC
symmetric
or
concentric
27
APICAL
Small
cavity
remains
Apical Hypertrophy
28
HYPERTROPHIC CARDIOMYOPATHY
CLINICAL PRESENTATIONS
Some patients asymptomatic
Symptomatic
dyspnea in 90%
angina pectoris in 75%
fatigue, pre-syncope, syncope
risk of SCD in children and adolescents
palpitation, PND, CHF, dizziness less frequent
Costello syndrome
ECG : giant negative T waves in the precordial leads
Spade like appearence
29
HYPERTROPHIC CARDIOMYOPATHY
PHYSICAL EXAMINATIONS
Carotid impulse
Prominent a waves of JV pulse
Outflow murmur
Mitral regurgitation
Atrial fibrilation
Embolic phenomena
Heart Failure symptoms
30
HYPERTROPHIC CARDIOMYOPATHY
DIAGNOSTIC APPROACH
ECG :
Abnormalities of ST segment and T waves
LVH
QRS complex tallest at mid precordial leads

Echo :
Concentric caused by pressure overload e.g. AS
Eccentric caused by volume overload e.g MR, AR
Septal thickening wall
Mitral valve anterior leafleat may be enlarged
MR


31
32
HYPERTROPHIC CARDIOMYOPATHY
DIFFERENTIAL DIANOSIS
Left Ventricular hypertrophy
Outflow obstruction secondary to valvular
heart disease e.g AS, coarctation of aorta and
infiltrative disorder of myocardium
Pattern hypertrophy in hypertension is
concentric meanwhile in HCM is distinctive
33
HCM vs Aortic Stenosis
HCM Fixed Obstruction

carotid pulse spike and dome parvus et tardus
murmur radiate to carotids
valsalva, standing
squatting, handgrip
passive leg elevation
systolic thrill 4th left interspace 2nd right interspace
systolic click absent present
34
Hypertrophic Cardiomyopathy
Treatment algorithm
35
HYPERTROPHIC CARDIOMYOPATHY
MANAGEMENT
Medical management:
B-blockers
CCB, verapamil
Disopyramide in decreasing outflow gradient
Endocarditis prophylaxis
Permanent Pacing
I C D
Alcohol ablation of the septum
Injected 1-4 ml absolute alcohol into the septal
perforator branch of LAD
36
HYPERTROPHIC CARDIOMYOPATHY
MANAGEMENT
Surgical therapy
Subaortic ventricular myotomy
Resection myocardium from proximal septum to
beyond mitral leafleat
Advantage :
Low mortality
Reduced symptoms
Improved functional capacity
Heart transplantation

37
Restrictive Cardiomyopathy
38
RESTRICTIVE CARDIOMYOPATHY
Hallmark: abnormal diastolic function
rigid ventricular wall with impaired
ventricular filling
importance lies in its differentiation from
operable constrictive pericarditis
Characteristics by : Abnormal compliance of
the left ventricle and short relaxation time

39
RESTRICTIVE CARDIOMYOPATHY
ETIOLOGY
Non infiltrative cause
Associated with patchy endomyocardial
fibrosis, increased cardiac mass and enlarged
atria
Infiltrative cause
Amyloidisis, primary caused by the deposition of
amyloid protein. Secondary caused by production
nonimunoglobulin protein and termed AA
Sarcoidosis
Endomyocardial fibrosis

40
RESTRICTIVE CARDIOMYOPATHY
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%
41
RESTRICTIVE CARDIOMYOPATHY
CLASSIFICATION
Idiopathic
Myocardial
1. Noninfiltrative
Idiopathic
Scleroderma
2. Infiltrative
Amyloid
Sarcoid
Gaucher disease
Hurler disease
3. Storage Disease
Hemochromatosis
Fabry disease
Glycogen storage
Endomyocardial
endomyocardial fibrosis
Hyperesinophilic synd
Carcinoid
metastatic malignancies
radiation, anthracycline
42
RESTRICTIVE CARDIOMYOPATHY CLINICAL
PRESENTATIONS
Symptoms of right and left heart failure
Angina if CAD involve
Jugular Venous Pulse
prominent x and y descents
Echo-Doppler
abnormal mitral inflow pattern
prominent E wave (rapid diastolic filling)
reduced deceleration time ( LA pressure)
43
RESTRICTIVE CARDIOMYOPATHY
DIAGNOSTIC APPROACH
ECG :
Low voltage
Poor R wave progression
Pseudoinfarction pattern in the inferior leads
P pulmonal If pulmonary hipertension present
Atrial arrhythmia esp fibrillation
Sick sinus syndrome is common
Ventricular Tachiarrhytmia
44
RESTRICTIVE CARDIOMYOPATHY
DIAGNOSTIC APPROACH
Chest X-ray
Normal CTR and enlarged atria
Enlarged right ventricle may be seen if pulmonary
hypertension present
Echo
Severe biatrial enlargement
Thickened LV walls
Cardiac nuclear imaging
CT scan and MRI
Cardiac catheterization and endomyocardial biopsy
45
No satisfactory medical therapy
Drug therapy must be used with caution
diuretics for extremely high filling prssures
vasodilators may decrease filling pressure
? Calcium channel blockers to improve diastolic
compliance
digitalis and other inotropic agents are not
indicated
RESTRICTIVE CARDIOMYOPATHY
MANAGEMENT
46
RESTRICTIVE CARDIOMYOPATHY
DIFFERENTIAL DIAGNOSIS
Constrictive pericarditis
Chronic RV infarction
RV dysfunction from RV pressure or RV
volume overload
Instrinsic RV myocardial disease
Tricuspid valve disease
47
Constrictive - Restrictive Pattern
Square-Root Sign or Dip-and-Plateau
48
49
RESTRICTIVE CARDIOMYOPATHY
Restriction vs Constriction
History provide can important clues
Constrictive pericarditis
history of TB, trauma, pericarditis, sollagen
vascular disorders
Restrictive cardiomyopathy
amyloidosis, hemochromatosis
Mixed
mediastinal radiation, cardiac surgery
50
MYOCARDITIS
Inflammation of myocardium
Can be result of systemic disorder or
infectious agent
Viral-Coxsackie B, echovirus, influenza,
parainfluenza, Epstein-Bar, and HIV
Bacterial-C. Diptheria, N. meningitidis, M.
pneumonia, and beta-hemolytic strep
Frequently accompanied with pericarditis
51
MYOCARDITIS
Clinical Presentations
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
52
MYOCARDITIS
CLINICAL PRESENTATIONS
EKG-nonspecific changes, av block, prolonged
QRS suration, or ST elevation(with pericarditis)
CXR-Normal
Cardiac Enzymes- may be elevated
Differentail-ischemia or infarct, valvular
disease, and sepsis
53
MYOCARDITIS
MANAGEMENT
Supportive care
Blood cultures
Antibiotics for bacterial cause
Watch for signs of progressive heart failure
54
Pericardial disease
55
Fibrous sac surrounding
heart-dense network of
collagen fibres
Serous membrane
two continuous layers
separated by a small
amount of fluid lubricant
(10-20mls )
Layers are :
Visceral is inner layer
(epicardium)
Parietal is continuous with
diaphragm and outer walls
of great arteries

56
ACUTE PERICARDITIS
Loose visceral pericardium and dense parietal
pericardium surround heart
Pericardial space may contain up to 50ml
normally
Etiologies of acute pericarditis-viral, bacterial,
fungal, malignancy, drugs, radiation,
connective tissue disorder, uremia,
myxedema, post-MI, or idiopathic
57
ACUTE PERICARDITIS
ETIOLOGY
Idiopathic 86%
Infective (viral or bacterial) 7%
Following a myocardial infarction or cardiac surgery
(Dresslers syndrome)
Radiation therapy
Neoplastic disease (commonly lung or breast) 6%
Connective tissue disease





Figures from Permanyer-Miralda et al 1985 58
ACUTE PERICARDITIS
CLINICAL PRESENTATIONS

Retrosternal chest
pain sharp or
stabbing pain worse
on insp and lying flat
Friction rub (high
pitched scratching
noise)
Raised jugular venous
pressure

59
ACUTE PERICARDITIS
DIAGNOSIS APPROACH
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
60
ACUTE PERICARDITIS
DIAGNOSIS APPROACH
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
61
ACUTE PERICARDITIS
MANAGEMENT
Search for the underlying disease
No good evidence from randomised controlled trials
Patients require bed rest
NSAID (aspirin, indomethacin) are generally accepted
as effective for relieving symptoms of chest pain
NSAID ketorolac tromethamine rapid results
Colchicine may be a useful adjunct in those who do
not respond to NSAIDs alone
62
ACUTE PERICARDITIS
PROGNOSIS
Pericarditis is usually a benign disorder
Diagnosis relates to underlying cause
But any cause can lead to an effusion and
tamponade which can lead to death
Pericarditis can also progress to pericardial
constriction and heart failure
63
CONSTRICTIVE PERICARDITIS
Occurs when fibrous thickening and loss of
elasticity interfere with diastolic filling
Cardiac trauma, pericardiotomy,
intrapericardial hemmorhage, fungal or
bacterial pericarditis, uremic pericarditis are
most common causes
64
CONSTRICTIVE PERICARDITIS
CLINICAL PRESENTATIONS
Sxs gradually develop-mimics restrictive CM-
CHF, DOE, and decreased exercise tolerance
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
65
CONSTRICTIVE PERICARDITIS
DIAGNOSIS APPROACH
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

66
CONSTRICTIVE PERICARDITIS
DIFFERENTIAL DIAGNOSIS
Consider acute pericarditis, myocarditis,
exacerbation of chronic ventricular
dysfunction, or systemic process resulting in
decreased cardiac performance(sepsis)
67
CONSTRICTIVE PERICARDITIS
MANAGEMENT
Supportive care
Symptomatic patients require admission and
pericardiectomy
68
PERICARDIAL EFFUSION
Major causes are post cardiac surgery and
Neoplastic disease
Gradual accumulation of fluid (chronic) permits
progressive stretching of pericardium
Patient may develop a substantial fluid without
significant increase in intrapericardial pressure
69
PERICARDIAL EFFUSION
PATOPHYSIOLOGY
Significantly increased intrapericardial pressure
impedes diastolic filling of the ventricles
Therefore in order for the ventricles to fill the
end-diastolic pressure must exceed the pericardial
pressure
Global effusion pericardial pressure is equal
around heart
Therefore both ventricles have to increase EDP to
same amount for ventricles to fill
70
PERICARDIAL EFFUSION
DIAGNOSTIC APPROACH
Clinical examination SOB, orthopnoea, tachycardia
(varying degrees)
Auscultation may have muffled heart sounds
ECG may show low amplitude QRS complexes and
alternating axis
CXR globular appearance to heart and therefore
increased cardiothoracic ratio
Echo size of effusion and haemodynamic effect of it
71
C X R ECHO
72
PERICARDIAL EFFUSION
TREATMENT

Depends on the cause and nature
If acute the cause is treated and the patient
monitored
If persistent problem or life threatening more
dramatic action is called for
73
CARDIAC TAMPONADE
Occurs when the fluid accumulation around the
heart impairs filling to such an extent that there is
haemodynamic compromise.
It is a medical emergency and must be treated
promptly.
Risk of death depends upon speed of diagnosis,
treatment and underlying cause of the tamponade.
74
CARDIAC TAMPONADE
CLINICAL PRESENTATIONS
Dyspnea and decreased exercise tolerance-wt
loss, pedal edema, ascites
Tachycardia, Narrow pulse pressure
Pulsus paradoxus
JVD, Muffled heart sounds, Hypotension
75
CARDIAC TAMPONADE
DIAGNOSTIC APPROACH
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 globular heart
ECHO :
- Size and location of effusion
- Any evidence of diastolic collapse
- Swinging of the heart
- Decrease of insp. flow across MV


76
CARDIAC TAMPONADE
DIAGNOSTIC APPROACH
IV Fluid Bolus-improves
RV filling and improves
hemodynamics
Pericardiocentesis-
therapeutic and
diagnostic
Admission to ICU or
monitored setting
77