CARDIOMYOPATHY
Rizki Amalia Gumilang
CARDIOMYOPATHIES
A group of heart disorders, major
structural abnormality is limited to the
myocardium
Often result Heart failure
etiology frequently unknown
Hyperthrophic
Dilated CM Restrictive CM
CM
Ventricular Thickened Stiffened
enlargement myocardium myocardium
Impaired systolic Abnormal diastolic Impaired diastolic
function function function
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DILATED CARDIOMYOPATHY
Dilated all 4 chamber (typical DCM)
Decrease contractile function (systolic)
Low CO
MR or TR or both
Arrythmia
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Etiology
Idiopathic
Inflammatory
Infection : Viral, bacterial, parasit
Noninfection : Sarcoidosis, peripartum CM
Toxic
Alkohol
Chemotherapeutic agent
Metabolic
Hypothyroidism
Chronic hypoCa or HypoPO4
Neuromuscular
Muscular or myotonic dystrophy
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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
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PATHOPHYSIOLOGY
MYOCYTE INJURY
Contractility
Pulmonary congestion
Dyspneu, orthopneu, rales
Fatigue
Systemic congestion Weakness
Edema, ascites, JVD
SV
Ventricular filling
LV Dilatation Forward CO
pressure
Mitral Regurgitasi
Lilly L.S, 2007, Pathophysiology of Heart Disease
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NEUROHUMORAL ACTIVATION
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CLINICAL PRESENTATIONS
Heart failure symptoms
Anginal chest pain
Emboli (systemic or pulmonary)
Syncope
Sudden cardiac death
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INVESTIGATION
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
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echocardiography
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treatment
THE GOAL THERAPY :
Treatment of underlying disease
Relieve
Treatment symptom
of heart failure
Prevent complication
Relief vascular congestion
Augmentation of low CO
Improve
Prevention long term survival
& treatment of arrhythmias
Prevention of thromboembolic event
Cardiac transplantation
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Hyperthrophic CARDIOMYOPATHY
LV hyperthrophy
Cardiac cause (-)
Systemic cause (-)
Small LV cavity
Contractile function is
vigorous
Impaired ventricular
relaxation
High diastolic pressure
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etiology
HCM is the most common genetic cardiovascular
disease.
Mutations of the cardiac sarcomere myofilaments
Affected genes :
-myosin heavy chain,
myosin-binding protein C
cardiac troponin T and I
-tropomyosin
actin
titin
myosin light chains.
inherited in an autosomal dominant
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pathology
Pathological changes are muscular
hyperthrophy associated with fibrosis
Muscle fiber hyperthrophy
Myocardial disarray some part of heart
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pathology
Normal muscle structure Myocardial disarray
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Types of hcm
HCM or HOCM
Asymmetric septal (ASH) - without obstruction
Asymmetric septal (ASH) - with obstruction
Symmetric hypertrophy - concentric
Apical hypertrophy
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pathophysiology
Myocyte Dynamic LVO
hyperthrophy obstruction
Ventricular Systolic
LVH pressure
arrhythmia
Mitral
regurgitation
LVDEP MVO2
Failure to CO
with exertion
Sudden
Syncope Dyspneu Angina
death
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INOTROPIC
Mitral valve
Venturi
presses against
effect
septum
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Clinical findings
Asymptomatic
Symptomatic :
Dyspnea
Angina
Presyncope / syncope
Palpitation due to arrhythmia
Sudden death young population
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Physical examination
Usually limited to HOCM patient
Carotid impulse
Prominent a waves of JV pulse
Palpable S4 double apical impulse
S4 can be heard
Outflow murmur
Mitral regurgitation
Atrial fibrilation
Heart Failure symptoms
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Murmur of as & hcm
VALSAVA SQUATTING STANDING
HCM murmur
AS murmur
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Diagnostic study
Electrocardiography
Chest X-ray
Echocardiography
Cardiac catheterization
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HCM - ECG
LAD
LVH, LAE
Prominent Q waves in inferolateral leads
ST-T changes
Arrhytmia AF, VT
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HCM - Cxr
Mild to moderate enlargement of the
cardiac silhouette
Concentric left ventricular hypertrophy
Enlargement of the left atrium and the
right
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HCM - echocardiography
2 Dimension & M-Mode :
LV wall thickening (15 mm)
Region
Left Ventricular Outflow Tract Obstruction (LVOTO)
Abnormal motion of Anterior Mitral Leaflet (AML)
Doppler :
Outflow pressure gradient
Associated Mitral Regurgitation (MR)
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Differential diagnostic
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
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Treatment drugs theraphy
blocker (standart therapy)
Myocardial O2 demand angina & dyspneu <<
LV outflow gradient
Passive diastolic filling time
Frequency of ventricular ectopic beat
Ca channel blocker
Reduce ventricular stiffness
Improve exercise capacity
Reduce rhytme disturbance
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Amiodarone and disopyramide
For arryhthmias
Reduce inotropic
Infective endocarditis prophylaxis
Diuretic
For pulmonary congestion (avoid volume depletion!!!)
Avoid !!!
Nitrates
Digitalis
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Treatment nondrugs theraphy
Septal myectomy
Dual chamber pacing
Decrease LVOTO
Reduce symptom
Alkohol septal ablation
Newer modality
Decrease LVOTO
Lessen systolic anterior motion (SAM) of mitral valve
Reduce LVOT gradient
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RESTRICTIVE CARDIOMYOPATHY
Restrictive filling
Normal or reduced
LV and RV volumes
Abnormal diastolic
function
Normal or nearly
normal systolic (LV
and RV) function
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etiology
Myocardial Endomyocardial
Non infiltratif Obliterative
Idiopathic Endomyocardial fibrosis
Scleroderma Hypereosinophilic syndrome
Infiltratif Nonobliterative
Amyloidosis Carcinoid
Sarcoidosis Malignant infiltration
Iatrogenic (radiation therapy)
Storage disease
Hemochromatosis
Glycogen storage diseases
Lilly L.S, 2007, Pathophysiology of Heart Disease
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Pathophysiology
Rigid myocard
JVD
Fatigue Hepato>>
Diastolic & Ascites
ventricular
Ventricular filling
Weakness Peripheral edema
pressure
Cardiac output Venous congestion
Lilly L.S, 2007, Pathophysiology of Heart Disease
40
Clinical findings
Fatigue
Decrease exercise tolerance
Pulmonary congestion
Systemic congestion (JVD, ascites, peripheral edema)
Kussmaul sign
S3 and S4 gallops
Arrhythmias
Various type of heart block
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DIAGNOSTIC APPROACH
CXR
Normal size ventricles
Pulmonary congestion
Atrial enlargement
ECG
Low voltage
Poor R wave progression
Pseudoinfarction pattern in the inferior leads
Nonspecific ST-T wave abnormalies
Conduction disturbances
Atrial and ventricular arrhythmias
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DIAGNOSTIC APPROACH cont.
Echocardiographic (nonspecific)
Exculpate other causes of HF
Abnormal diastolic function
Endomyocardial biopsy
Cardiac MRI
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management
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
Chronic oral anticoagulant
Antiarrhythmias
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