This document provides information on various types of cardiomyopathy. It defines cardiomyopathy as diseases that primarily affect the heart muscle and are not caused by other structural or vascular issues. The main types discussed are dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy. Causes, signs and symptoms, diagnostic criteria, classification systems, treatment approaches, and prognosis are described for each type.

2.  DEFINITION of CARDIOMYOPATHY---
 These are a group of diseases that primarily affect the
heart muscle and are NOT the result of
congenital/acquired valvular/hypertensive/coronary
arterial/pericardial abnormalities…

3.  This group comprises of---

 1. Dilated cardiomyopathy..
 2. Hypertrophic ………..
 3. Restrictive …………..
 4. Acute myocarditis…

4.  TWO FUNDAMENTAL FORMS are there---
 1. PRIMARY Type… (consists of heart muscle
disease predominantly involving the myocardium)..
 2. SECONDARY Type.. (consists of myocardial
disease of known cause or associated with a systemic
disease )….

5. WHO Classification
 Unknown cause
(primary)
 Dilated
 Hypertrophic
 Restrictive
 unclassified
 Specific heart muscle
disease (secondary)
 Infective
 Metabolic
 Systemic disease
 Heredofamilial
 Sensitivity
 Toxic
Br Heart J 1980; 44:672-673

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Cardiomyopathy
Cardiomyopathy
Nursing Review, 2001

7. Functional Classification
 Dilated (Congestive, DCM, IDC)
 Ventricular dilation, hypokinetic left ventricle, and systolic
dysfunction
 Hypertrophic (IHSS, HCM, HOCM, ASH)
 Inappropriate myocardial hypertrophy, with or without left ventricular
obstruction
 Restrictive (Infiltrative)
 Abnormal ventricular filling with diastolic dysfunction
 Arrhthymogenic Right Ventricular (ARVD)
 Fibroadipose replacement of right ventricle
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8. Dilated Cardiomyopathy
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9. Dilated Cardiomyopathy -
Definition
 Primary (idiopathic) is a disease of unknown etiology that
principally affects the myocardium leading to LV dilation and
systolic dysfunction
 Secondary causes include ischemia, alcoholic, peripartum, post-
infectious, viral
 Most common of the cardiomyopathies
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10. Schematic of Dilated CM
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Medslides.com

11. DCM - Incidence and Prognosis
 Prevalence is 36 per 100,000 population
 Third most common cause of heart failure
 Most frequent cause of heart transplantation
 DCM accounts for approximately 10,000 deaths and 46,000
hospitalizations per year in the US
 Complete recovery is rare
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Source: UpToDate (www.uptodate.com) "Definition and classification of the cardiomyopathies“ Leslie T
Cooper, Jr, MD last updated 2/14/08

12. Idiopathic Dilated
Cardiomyopathy
104
72
56
51
45
37 35 31
24
19 16
0
20
40
60
80
100
120
0 1 2 3 4 5 6 7 8 9 10
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Years
Am J Cardiol 1981; 47:525
Observed Survival of 104 Patients

13.  Genetic considerations in DCMP–
 Most common mutations are those found in genes
encoding sarcomeric proteins,such as @ cardiac
actin,b and @ myosin,and Troponins T,I and C….
 Its postulated that abnormal proteins cause contractile
dysfunction by impairing the production or
transmission of force…

14. Clinical Manifestations
 Highest incidence in middle age
 Blacks 2x more frequent than whites
 Men 3x more frequent than women
 Symptoms may be gradual in onset
 Acute presentation
 Misdiagnosed as viral URI in young adults
 Uncommon to find specific myocardial disease on
endomyocardial biopsy
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Source: UpToDate (www.uptodate.com) "Clinical manifestations and evaluation of the patient with
suspected heart failure" Wilson S Colucci, MD, last updated 6/7/09

15. Clinical Manifestations (Cont)
 Symptoms/Signs of heart failure
 Pulmonary congestion (left heart failure)
dyspnea (rest, exertional, nocturnal), orthopnea
 Systemic congestion (right heart failure)
edema, nausea, abdominal pain, nocturia
 Low cardiac output
 Hypotension, tachycardia, tachypnea
 Fatigue and weakness
 Arrhythmia
 Atrial fibrillation, conduction delays, complex PVC’s, sudden
death
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16. Diagnostics
 CXR (enlarged heart, CHF)
 Electrocardiogram (tachycardia, A-V block, LBBB, NSSTT changes,
PVC’s)
 24-hour Holter monitor
 if lightheadedness, palpitation, syncope
 Echocardiogram (left ventricular dilation, global hypokinesis, low
EF)
 Myocardial biopsy, rare
 Cardiac catheterization (R/O CAD)
 if age >40, ischemic history, high risk profile, abnormal ECG
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17. Clinical Indications for
Endomyocardial Biopsy
 Definite
 monitoring of cardiac allograft rejection
 monitoring of anthracycline cardiotoxicity
 Possible
 detection and monitoring of myocarditis
 diagnosis of secondary cardiomyopathies
 differentiation between restrictive and constrictive heart
disease

18.  ALCOHOLIC CARDIOMYOPATHY–
 1. Ingestion of alcohol > 90 g/d of alcohol over many
years may result in DCMP…. (SECONDARY)..
 2. Role of gene polymorphism encoding the alcohol
metabolizing enzyme , ALCOHOL
DEHYDROGENASE and of DD form of ACE gene is
there…
 3. Management consists of abstinence , which may
halt the progression or even reverse the course of
this disease…

19.  HOLIDAY HEART SYNDROME---
 It typically appears after a drinking binge,AF is seen
most frequently, followed by atrial flutter and
frequent VPCs…

20.  NOTE–
In contrast to the adverse cardiac effects of excessive
alcohol consumption,moderate consumption (20-30
g/d)appear to be cardioprotective…
 It raises HDL cholesterol and is associated with a
reduced incidence of IHD,Ischemic stroke and
metabolic syndrome….

21.  ISCHEMIC CARDIOMYOPATHY---
 The diffuse myocardial fibrosis that accompanies
multiple myocardial scars produced by myocardial
infarctions , can impair LV function and is referred as
ISCHEMIC CARDIOMYOPATHY….

22. PERIPARTUM CARDIOMYOPATHY-
---
 1. Cardiac dilatation and CHF may develop during
the last trimester of pregnancy /within 6
months of delivery….
 2. Exact cause is unknown…
 3. The typical patient profile is a multiparous
woman,aged> 3o years…
 4. All the symptoms/signs/treatment are the same
as DCMP…..
 5. Mortality rate is 10%.....

23.  6. The prognosis is related to whether the heart
size returns to normal after the first episode of
CHF….
 However,if the heart size remains enlarged,and the
LVEF remains depressed,the prognosis is poor….
 7. Patients,who recover from peripartum
cardiomyopathy should be encouraged to avoid
further further pregnancies,particularly if the
LV dysfunction persists….

24. DCM - Treatment
 Limit activity based on functional status
 Salt restriction
 Fluid restriction
 Initiate medical therapy
 ACE inhibitors, diuretics
 Digoxin, coreg
 Hydralazine/nitrate combination
 Anticoagulation prn (EF <30%, hx of embolic events)
 Implantable defibrillators
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25. DCM – Treatment (Cont)
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 Cardiac transplantation
 This disorder is the most common indication for cardiac
transplantation
 Survival after transplant is
 80% one year
 70% 5 years
 Left Ventricular Reduction Procedures
 LV-reshaping
Source: UpToDate (www.uptodate.com) "Diagnosis and management of ischemic cardiomyopathy" James
C Fang MD, Sary Aranki MD, last updated 4/7/09

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Restrictive Cardiomyopathies
• Hallmark: abnormal diastolic function
• Rigid ventricular wall with impaired ventricular filling
• Bear some functional resemblance to constrictive pericarditis
• Importance lies in its differentiation from operable constrictive pericarditis
• Much less common then DCM or HCM outside the tropics, but frequent
cause of death in Africa, India, South and Central America and Asia
primarily because of the high incidence of endomyocardial fibrosis in those
regions

27.  Idiopathic
 Myocardial
 Noninfiltrative
 Idopathic
 Scleroderma
 Infiltrative
 Amyloid
 Sarcoid
 Gaucher disease
 Hurler disease
 Storage Disease
 Hemochromatosis
 Fabry disease
 Glycogen storage
 Endomyocardial
 Endomyocardial fibrosis
 Hyperesinophilic synd
 Carcinoid
 Metastatic malignancies
 Radiation, anthracycline
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Classification

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Clinical Manifestations
• Symptoms of right and left heart failure
• Thrombo-embolic complications…
• Kussmaul’sign is positive… ;3rd and 4th heart sounds may be
there…
• Jugular Venous Pulse
• Echo-Doppler
– Abnormal mitral inflow pattern
– Prominent E wave (rapid diastolic filling)
– Reduced deceleration time ( LA pressure)

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Restriction vs. Constriction
• History can provide important clues
– Constrictive pericarditis
• History of TB, trauma, pericarditis, collagen vascular disorders
– Restrictive cardiomyopathy
• Amyloidosis, hemochromatosis
– Mixed
• Mediastinal radiation, cardiac surgery

30.  NOTE--
 In contrast to Constrictive pericarditis,which RCM
resembles in many respects,the apex impulse is
usually easily palpable ,and MR is more common…

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Treatment
• No satisfactory medical therapy
• Drug therapy must be used with caution
– Diuretics for extremely high filling pressures
– Vasodilators may decrease filling pressure
– (?) Calcium channel blockers to improve diastolic compliance
– Digitalis and other inotropic agents are not indicated

32. Arrhythmogenic RV
Cardiomyopathy
 Characterized by fibroadipose replacement of segments of the free
wall of the right ventricle
 Familial and progressive
 Predominately found in young adults
 Cause of young adult sudden death
 ICD implantation in ALL patients who are symptomatic with
arrhythmias
 ICD implantation vs anti-arrhythmic meds in asymptomatic
patients?
 Prognosis????
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Hypertrophic Cardiomyopathy
(HCM)
 Defined
 First described in 1869 and accepted as a clinical entity in the 1950’s
 Prevalence 2:1000
 Genetic disease characterized by hypertrophy of the left ventricle
with marked variable clinical manifestations morphologic and
hemodynamic abnormalities
 Small LV cavity, septal hypertrophy which can be asymmetric
(ASH), systolic anterior motion of the mitral valve leaflet (SAM),
+/- obstruction of left ventricular outflow with low stroke volume,
but elevated EF
Source: UpToDate (www.uptodate.com) “Natural history of hypertrophic cardiomyopathy" Perry M
Elliott, MD, William J McKenna, MD, last updated 9/18/07

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65 -
70%
8 - 10% 15 - 20%
www.kanter.com/hcm
Variants of HCM

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HCM – ASH Without
Obstruction
 The major abnormality of the heart in HCM
is an excessive thickening of the muscle.
Thickening usually begins during early
adolescence and stops when growth has
finished. It is uncommon for thickening to
progress after this age
 The left ventricle is almost always affected,
and in some patients the muscle of the right
ventricle also thickens
 Hypertrophy is usually greatest in the
septum. The muscle thickening in this
region may be sufficient to narrow the
outflow tract. This thickening is associated
with obstruction to the flow of blood out of
the heart into the aorta
www.kanter.com/hcm

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HCM – ASH With Obstruction
 Asymmetric septal hypertrophy
with obstruction to the outflow of
blood from the heart may occur. The
mitral valve touches the septum,
blocking the outflow tract. Some
blood is leaking back through the
mitral valve causing mitral
regurgitation
www.kanter.com/hcm

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Pathophysiology of HCM
 Dynamic LV outflow tract obstruction
 Outflow tract gradient (>30 mm Hg), considered severe if >50 mm Hg
(occurs in 25-30% of cases leading to name hypertrophic obstructive
cardiomyopathy)
 Diastolic dysfunction
 Impaired diastolic filling,  filling pressure
 Myocardial ischemia
 Mitral regurgitation
 Arrhythmias

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Left Ventricular Outflow Tract
Gradient
 Approximately 25% of patients with HCM have a dynamic systolic
pressure gradient in the left ventricular outflow tract caused by
contact between the mitral valve leaflet(s) and the interventricular
septum under resting conditions
 Outflow tract gradient in excess of 30 mmHg is an important cause
of symptoms
 Question if gradient is simply a consequence of high velocity flow
through the aortic valve, and hence does not represent a real
obstruction to cardiac output - controversy
Source: UpToDate (www.uptodate.com) "Pathophysiology of obstructive hypertrophic cardiomyopathy"
William J McKenna, MD, last updated 12/29/08

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Left Ventricular Outflow Tract Gradient (Cont)
 Gradient greater than 50 mmHg, the percentage of systolic volume
ejected before the beginning of SAM is greatly reduced -
responsible for patients' symptoms when severe, outflow tract
gradient can cause dyspnea, chest pain, syncope, and predisposes
to the development of atrial arrhythmias - independent predictor
of disease progression and adverse outcome, including sudden
death

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Clinical Manifestations
 Asymptomatic
 Echocardiographic finding only
 Symptomatic
 Dyspnea in 90%
 Angina pectoris in 75%
 Fatigue, pre-syncope, syncope, risk of SCD
 Palpitation, PND, CHF, dizziness
 Atrial fibrillation, thromboembolism

42.  PHYSICAL EXAMINATION---
 1. Most patients demonstrate double or tripal apical
precordial impulse… and a 4th heart sound..
 2. Systolic murmur,which is typically harsh,diamond
shaped,and usually begins after S1…
 3. This murmur is best heard at the lower left sternal
border as well as at the apex…

43.  DYNAMIC AUSCULTATION---
 The pressure gradient in HOCM is dynamic and
changes with various manuvers…
 3 Basic mechanisms involved are---
 a. Increased LV contractility
 b. Decreased Ventricular preload
 c. Decreased aortic impedance and
pressure(afterload)…..

44.  1. Interventions,that increase myocardial
contractility,such as exercise and sympathomimetic
amines causes an increase in the gradient and the
murmur…..
 2. The same is the result,when interventions , that
reduce preload,such as the strain phase of the
Valsalva maneuver,sudden standing,or nitroglycerin
are done….
 3. Conversely,elevation of arterial pressure by
squatting,sustained handgrip,augmentation of venous
return by passive leg raising all increase
ventricular volume and ameliorate the gradient and
murmur…..

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EKG Findings
 Abnormal in 85-90% of cases
 LVH, Strain pattern
 Abnormal ST-T’s, giant T wave inversions
 Abnormal Q’s,
 Bundle Branch Block
 Left atrial enlargment
 Ventricular arrhthymias
Source: UpToDate (www.uptodate.com) "Clinical manifestations and diagnosis of arrhythmogenic right
ventricular cardiomyopathy“ William J McKenna, MD, last updated 3/18/09

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Echocardiogram
 Left ventricular hypertrophy >1.3 cm (usually >1.5 cm)
 Septal to posterior wall ratio >1.3:1
 Mitral regurgitation
 Systolic anterior motion of the mitral valve (SAM)
 Premature midsystolic closure of the aortic valve
 Asymmetric septal hypertrophy (ASH)
 Diastolic dysfunction
 Left ventricular outflow tract obstruction (LVOT)

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Echocardiogram
 LVH usually develops between 5-15 years of age in HCM
 A normal ECHO in a young child does not R/O the diagnosis
 Serial ECHOs are recommended up to the age of 20 yr where there
is a family history of HCM
Source: UpToDate (www.uptodate.com) "Clinical manifestations and diagnosis of arrhythmogenic right
ventricular cardiomyopathy“ William J McKenna, MD, last updated 3/18/09

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Natural History & Clinical Course
 Clinical presentation from infancy to old age
 Variable clinical course 25 % of cohort achieve normal longevity
 Annual mortality 3% in referral centers probably closer to 1% for all
patients
 Course may be punctuated by adverse clinical events: sudden cardiac
death, embolic stroke, and consequences of heart failure
 Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden
death

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Natural History & Clinical Course
 Risk of SCD higher in children, may be as high as 6% per year,
majority have progressive hypertrophy
 Accounts for 36% of deaths in athletes <35 years
 Clinical deterioration usually is slow
 Poor prognosis in males, young age of onset, family Hx of SCD,
Hx of syncope, exercise induced hypotension (worst)
 Progression to DCM occurs in 10-15%
Source: UpToDate (www.uptodate.com) “Natural history of hypertrophic cardiomyopathy" Perry M
Elliott, MD, William J McKenna, MD, last updated 9/18/07

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Risk Factors For SCD
 Young age (<35 years)
 “Malignant” family history of sudden death
 Aborted sudden cardiac death
 Sustained VT or SVT
 Non-sustained VT on holter monitoring
 Atrial fibrillation
 Dilated left ventricle
 NYHA classes III or IV

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Risk Factors For SCD
 Syncope
 Severe hypertrophy (>3.0 cm)
 Abnormal BP response to exercise
 Coronary artery disease
 Strenuous exercise or work

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Recommendations for Athletic
Activity
 Low-risk older patients (>30 years) may participate in athletic
activity if all of the following are absent:
 Ventricular tachycardia on Holter monitoring
 Family history of sudden death due to HCM
 History of syncope
 Severe hemodynamic abnormalities, gradient 50 mmHg
 Exercise induced hypotension
 Moderate or severe mitral regurgitation
 Enlarged left atrium (5.0 cm)
 Paroxysmal atrial fibrillation
 Abnormal myocardial perfusion

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Management of HCM
 Beta-adrenergic blockers (atenolol, metoprolol, sotalol .. etc)
 Calcium channel blockers (verapamil,diltiazem)…
 Anti-arrhythmics – Amiodarone, norpace
 Pacemakers (ICD)
 Myomectomy (resection of septum)
 Alcohol septal ablation (controlled MI through septal perforator
perfusing basal septum) wall thinningdecreases in LVOTO
 Transplantation