2KULIAH KARDIOMIOPATI

8/11/2019 2KULIAH KARDIOMIOPATI

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CARDIOMYOPATHY

Saharman Leman

Sub-bagian Kardiologi Penyakit Dalam Fk Unand /

Intensive Care Unit RSI Siti Rahmah Padang


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Definition

Disorders of the cardiac muscle of unknown

aetiology

A primary disorder of the heart musclethatcauses abnormal myocardial performance

and is not the result of disease or

dysfunction of other cardiac structures

myocardial infarction, systemic hypertension,

valvular stenosis or regurgitation


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Functional Classification

Dilatated (congestive, DCM, IDC)

ventricular enlargement and syst dysfunction

Hypertrophic (IHSS, HCM, HOCM) inappropriate myocardial hypertrophyin the absence of HTN or aortic stenosis

Restrictive (infiltrative)

abnormal filling and diastolic function


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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


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ETIOLOGIES OF DILATED CARDIOMYOPATHY

0

5

10

15

20

2530

35

40

45

50

Disorder

IDCM

Myocarditis

Ischmic CM

InfiltrativediseasePeripartum CM

Hypertension

HIVCTD

Substance

abuse

Felker et al NEJM 2000


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1. 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


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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 Ca2+-ATPase

Myofibrillar ATPase

Creatine Kinase


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MOLECULAR DEFECTS IN DILATED

CARDIOMYOPATHY

Fatkin D, et al. NEJM 1999;341

GENESLamin A/C

-sarcoglycan

Dystrophin

Desmin

Vinculin

Titin

Troponin-T

-tropomyosin

-myosin heavy chain

Actin

Mitochondrial DNAmutations


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CLINICAL PRESENTATIONS

Heart failure symptoms 75%-85%

Anginal chest pain 8%-20%

Emboli (systemic or pulmonary) 1%-4%

Syncope


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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

ventriculographyto assess : LV ejection fraction,end-diastolic volume, diastolic function

Cardiac catheterization

age >40, ischemic history, high risk profile,

abnormal ECG


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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, femalesex

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


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PREDICTING PROGNOSIS

Predictive Possible Not Predictive

Clinical factors symptoms alcoholism age

peripartum duration

family history viral illness

Hemodynamics LVEF LV sizeCardiac 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


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RIGHT

VENTRICULAR

BIOPSY

TECHNIQUE

ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY


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INDICATIONS FOR ENDOMYOCARDIAL

BIOPSY

Acute dilated cardiomyopathy with refractory heart failuresymptoms

Rapidly progressive ventricular dysfunction in anunexplained cardiomyopathy of recent onset

New onset cardiomyopathy with recurrent ventriculartachycardia or high grade heart block

Heart failure in the setting of fever, rash, and peripheraleosinophilia

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


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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


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MANAGEMENT

consider adding -blocking agents if

symptoms persists

anticoagulation for EF


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2. 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)


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HYPERTROPHIC CARDIOMYOPATHYEPIDEMIOLOGY

Prevalence : 1 per 500inUS

Higher in Black individual

Inherited in an autosomal

dominant pattern Predominance in younger

Male

Commonest cause of

sudden death duringexertion in young people


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HYPERTROPHIC CARDIOMYOPATHYPATHOGENESIS

Misconception that outflow tract

obstruction

Impaired ventricular compliance as

consequence of inappropriate myocardial

hypertrophy


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HYPERTROPHIC CARDIOMYOPATHYPATOPHYSIOLOGY

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


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HYPERTROPHIC CARDIOMYOPATHYMYOCARDIAL DISARRAY

Normal Muscle Structure Myocardial Disarray

(Parallel alignment) (Disorganised alignment)


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HYPERTROPHIC CARDIOMYOPATHYTYPES

HCM or HOCM

Asymmetric septal (ASH) - withoutobstruction

Asymmetric septal (ASH) - with obstruction

Symmetric hypertrophy - concentric Apical hypertrophy


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65% 35%

10%

www.kanter.com/hcm


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HCM

Mitral

Valve in

normal

position


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HOCM

Mitral valve

presses

against

septum

MR


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SYMMETRIC

symmetric

or

concentric


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APICAL

Small

cavityremains

Apical Hypertrophy


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HYPERTROPHIC CARDIOMYOPATHYPHYSICAL EXAMINATIONS

Carotid impulse

Prominent a waves of JV pulse

Outflow murmur Mitral regurgitation

Atrial fibrilation

Embolic phenomena Heart Failure symptoms


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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


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HYPERTROPHIC CARDIOMYOPATHYDIFFERENTIAL DIANOSIS

Left Ventricular hypertrophy

Outflow obstruction secondary to valvularheart 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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HCM vs Aortic Stenosis

HCM Fixed Obstruction

carotid pulse spike and dome parvus et tardus

murmur radiate tocarotids

valsalva, standing

squatting, handgrip

passive leg elevation

systolic thrill 4th left interspace 2nd right

interspace

systolic click absent present


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Hypertrophic Cardiomyopathy

Treatment algorithm


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HYPERTROPHIC CARDIOMYOPATHYMANAGEMENT

Medical management:

B-blockers

CCB, verapamil Disopyramide in decreasing outflow gradient

Endocarditis prophylaxis

Permanent Pacing

I C DAlcohol ablation of the septum

Injected 1-4 ml absolute alcohol into the septalperforator branch of LAD


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HYPERTROPHIC CARDIOMYOPATHYMANAGEMENT

Surgical therapy

Subaortic ventricular myotomy

Resection myocardium from proximal septum tobeyond mitral leafleat

Advantage :

Low mortality

Reduced symptoms Improved functional capacity

Heart transplantation


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3. Restr ic t ive Card iomyopathy


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RESTRICTIVE CARDIOMYOPATHY

Hallmark: abnormal diastolic function

rigid ventricular wall with impaired

ventricular filling

importance lies in its differentiation from

operable constrictive pericarditis

Characteristics by :Abnormalcompliance of the left ventricle and

short relaxation time


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RESTRICTIVE CARDIOMYOPATHYETIOLOGY

Non infiltrative cause

Associated with patchy endomyocardial fibrosis,

increased cardiac mass and enlarged atriaInfiltrative cause

Amyloidisis, primary caused by the deposition ofamyloid protein. Secondary caused by production

nonimunoglobulin protein and termed AASarcoidosis

Endomyocardial fibrosis


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RESTRICTIVE CARDIOMYOPATHYEXCLUSION 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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RESTRICTIVE CARDIOMYOPATHYCLASSIFICATION

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


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RESTRICTIVE CARDIOMYOPATHYCLINICAL PRESENTATIONS

Symptoms of right and left heart failure

Angina if CAD involve

Jugular Venous Pulse prominentxand ydescents

Echo-Doppler abnormal mitral inflow pattern

prominent E wave (rapid diastolic filling) reduced deceleration time (LA pressure)


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RESTRICTIVE CARDIOMYOPATHYDIAGNOSTIC 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


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RESTRICTIVE CARDIOMYOPATHYDIAGNOSTIC APPROACH

Chest X-ray

Normal CTR and enlarged atria

Enlarged right ventricle may be seen if pulmonaryhypertension present

Echo

Severe biatrial enlargement

Thickened LV walls

Cardiac nuclear imagingCT scan and MRI

Cardiac catheterization and endomyocardial biopsy


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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 CARDIOMYOPATHYMANAGEMENT


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RESTRICTIVE CARDIOMYOPATHYDIFFERENTIAL DIAGNOSIS

Constrictive pericarditis

Chronic RV infarction RV dysfunction from RV pressure or RV

volume overload

Instrinsic RV myocardial disease Tricuspid valve disease


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Constrictive - Restrictive Pattern

Square-Root Sign or Dip-and-Plateau


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RESTRICTIVE CARDIOMYOPATHYRestriction vs Constriction

History provide can important clues

Constrictive pericarditis

history of TB, trauma, pericarditis, sollagenvascular disorders

Restrictive cardiomyopathy

amyloidosis, hemochromatosis Mixed

mediastinal radiation, cardiac surgery


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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


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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


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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, valvulardisease, and sepsis


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MYOCARDITIS

MANAGEMENT

Supportive care

Blood cultures Antibiotics for bacterial cause

Watch for signs of progressive heart

failure


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Pericardial disease

Fib di


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Fibrous sac surrounding

heart-dense network of

collagen fibres

Serous membranetwo 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


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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 pericarditis-viral,

bacterial, fungal, malignancy, drugs,

radiation, connective tissue disorder,uremia, myxedema, post-MI, or idiopathic


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ACUTE PERICARDITISETIOLOGY

Idiopathic86%

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


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ACUTE PERICARDITISCLINICAL PRESENTATIONS

Retrosternal chestpainsharp orstabbing pain worseon insp and lyingflat

Friction rub (highpitched scratchingnoise)

Raised jugularvenous pressure


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ACUTE PERICARDITISDIAGNOSIS 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

C C S


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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


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ACUTE PERICARDITISMANAGEMENT

Search for the underlying disease

No good evidence from randomised controlledtrials

Patients require bed rest

NSAID (aspirin, indomethacin) are generallyaccepted as effective for relieving symptoms ofchest pain

NSAID ketorolac tromethamine rapid results Colchicine may be a useful adjunct in those who

do not respond to NSAIDs alone


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ACUTE PERICARDITISPROGNOSIS

Pericarditis is usually a benign disorder

Diagnosis relates to underlying cause

But any cause can lead to an effusion andtamponade which can lead to death

Pericarditis can also progress to

pericardial constriction and heart failure


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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


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CONSTRICTIVE PERICARDITISCLINICAL PRESENTATIONS

Sxs gradually develop-mimics restrictiveCM- 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 soundmay be heard at apex


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CONSTRICTIVE PERICARDITISDIAGNOSIS 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


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CONSTRICTIVE PERICARDITISDIFFERENTIAL DIAGNOSIS

Consider acute pericarditis, myocarditis,

exacerbation of chronic ventriculardysfunction, or systemic process resulting

in decreased cardiac performance(sepsis)


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CONSTRICTIVE PERICARDITISMANAGEMENT

Supportive care

Symptomatic patients require admission

and pericardiectomy


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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


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PERICARDIAL EFFUSIONPATOPHYSIOLOGY

Significantly increased intrapericardialpressure impedes diastolic filling of theventricles

Therefore in order for the ventricles to fill theend-diastolic pressure must exceed thepericardial pressure

Global effusionpericardial pressure is equalaround heart

Therefore both ventricles have to increaseEDP to same amount for ventricles to fill


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PERICARDIAL EFFUSIONDIAGNOSTIC APPROACH

Clinical examinationSOB, orthopnoea,tachycardia (varying degrees)

Auscultationmay have muffled heart sounds

ECG may show low amplitude QRS complexesand alternating axis

CXRglobular appearance to heart andtherefore increased cardiothoracic ratio

Echosize of effusion and haemodynamiceffect of it

C X R ECHO


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C X R ECHO



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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


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Occurs when the fluid accumulation around theheart impairs filling to such an extent that there

is haemodynamic compromise.

It is a medical emergency and must be treatedpromptly.

Risk of death depends upon speed of diagnosis,

treatment and underlying cause of the

tamponade.

CARDIAC TAMPONADE


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CARDIAC TAMPONADECLINICAL PRESENTATIONS

Dyspnea and decreased exercise

tolerance-wt loss, pedal edema, ascites

Tachycardia, Narrow pulse pressure

Pulsus paradoxus

JVD, Muffled heart sounds, Hypotension


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CARDIAC TAMPONADEDIAGNOSTIC APPROACH

EKG-low voltage QRS with ST elevation and PRdepression 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

CARDIAC TAMPONADE


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CARDIAC TAMPONADE

DIAGNOSTIC APPROACH

IV Fluid Bolus-

improves RV filling and

improveshemodynamics

Pericardiocentesis-

therapeutic and

diagnostic

Admission to ICU or

monitored setting


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Terima Kas ih

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