mf3 - cardiomyopathy

7/30/2019 MF3 - Cardiomyopathy

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CARDIOMYOPATHY

April Grace A. Chiongbian


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Group of diseases that primarily affect the

heart muscle.

Not a result of congenital, acquired, valvular,

hypertensive, coronary arterial or pericardial

abnormalities


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Three Morphologic Types

Dilated Cardiomyopathy

Restrictive Cardiomyopathy

Hypertrophic Cardiomyopathy


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EPIDEMILOGY

Endomyocardial Fibrosis

Children and young adults

Tropical and subtropical Africa

Frequent cause of CHF in Africa

Dilated Cardiomyopathy in Africa

Infection with Toxoplasma gondii and Coxsackievirus B

(Nigeria)

African Trypanosomiasis (Cameroon)

of patient hospitalized with CHF


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Peripartum DCM

More common in Africa than in North Aerica and

Europe

Low socio-economic Status

High Parity

Excessive Salt intake

Selenium deficiency

Prolonged lactation


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Commonly presents in 3rd to 4th decade of life

Cardiomyopathy in Asia

Thiamine Deficiency

Wet beri-beri heart disease

Selenium Deficiency

Keshans Disease\


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DILATED CARDIOMYOPATHY


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Ventricular chamber enlargement

Systolic pump function is impaired leading toprogressive cardiac dilatation


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Etiology

Familial

End result of myocardial damage due to

known or unknown Infectious, metabolic or

toxic agents

Late consequence of acute viral myocarditis

Reversible form may be found with alcohol

abuse, pregnancy, thyroid disease, cocaine use

and chronic uncontrolled tachycardia


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Alcoholic Cardiomyopathy

Peripartum Cardiomyopathy

Neuromuscular Diseases Drugs

Arrhythmogenic Right Ventricular

Cardiomyopathy/ Dysplasia (ARVC/D)


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Clinical Features

Symptoms develop gradually

LV dilatation occurs for months or years before

becoming symptomatic

Vague chest pain

Syncope due to arrythmias and systemic

embolism


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Physical Examination

Narrow pulse pressue

Jugular venous pressure is elevated

Third and fourth heart sounds Mitral or tricuspid regurgitation


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Laboratory Examination

Chest Roegenogram enlargement of cardiac

sillhouette

ECG left bundle branch block and ST-T wave

abnormality

CTI and CMRI LV dilatation

Cardiac Catheterization

Coronary angiography dilated, diffusely

hypokinetic left ventricle


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ALCOHOLIC CARDIOMYOPATHY

>90g/d of alcohol

Risk of developing cardiomyopathy is partially

determined genetically

Polymorphism of the gene encoding alcohol

dehydrogenase as well as the DD form of ACE

gene

Poor prognosis (


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Holiday Heart Syndrome Second presentation of alcoholic cardiotoxicity

Recurrent supraventricular or ventricular

tachyarrhythmias Appears after a drinking binge

Atrial fibrillation

Atrial flutter

Frequent ventricular premature depolarizations


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PERIPARTUM CARDIOMYOPATHY

Last trimester of pregnancy or within 6

months of delivery

Cause: Inflammatory myocarditis, immune

activation and gestational hypertension

Multiparous, African ancestry, >30 years old

Prognosis:

Poor: LV remains enlarged and/ or LV ejection

fraction remains depressed after 6 months


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NEUROMUSCULAR DISEASE

In Duchennes progressive muscular

dystrophy, mutations in genes that encode

dystrophin lead to mycocyte death

Tall R waves in right precordial leads with an

R/S ration >1.0, often associated with deep Q

waves in limb and lateral precordial leads

Supraventricular and ventricular arrhythmias


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Myocardial Dystrophy

Disorders of impulse formationn and AV

conduction

Syncope ad sudden death are major haards

Insertion of an ICD and/or permanent pacemaker


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DRUGS

Anthracycline derivatives

Systolic dysfunction and ventricular arrhythmias

Cardiotoxicity 3 months after last dose

Late contractile dysfunction Prevention: document preclinical deterioration of LV

function

Modification of dose and dose schedule

Take along with cardioprotective agents (iron-chelatordexrazoxone)

ACE inhibitors


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Trastuzumab

Cyclophosphamide

Imatinib mesylate Cocaine abuse

Cardiac complications: SCD, Myocarditis, DCM and

acute MI

Nitrates, calcium channel blockers, antiplatelet

agents and benzodiazepines


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Arrhythmogenic Right Ventricular

Cardiomyopathy/ Dysplasia (ARVC/D)

Familial

Progressive fibrofatty replacement of the right

ventricle and LV myocardium

Autosomal dominant

Mutations in desmosomes

Plakophilin-2 most common


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RV failure with Jugular venous distention

Hepatomegaly

Edema

Manifest during 2nd decade Ventricular tachyarrhythmias

RV failure

ECG

QRS prolongation

CT-Scan/ MRI

RV dilatation, RV aneurysm ad Fatty replacement

MANAGEMENT:

Restriction from Competitive sports

Antiarrhythmic therapy with beta blockers or amiodarone

TAKO TSUBO (stress)


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TAKO-TSUBO (stress)

CARDIOMYOPATHY

Apical Ballooning syndrome Abrupt onset of severe chest discomfort

Preceded by very stressful emotional or physical

event

Women >50 y/o

ST segment elevations; Deep T wave inversions

Severe akinesia of LV

Troponins mildly elevated

Reversible within 3-7 days

Adrenergic Surge involved mechanism


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LEFT VENTRICULAR NONCOMPACTION

Uncommon congenital

Present at any age (CHF, thromboembolism,

Ventricular arrhythmias)

Arrest of normal embryogeneis

Echocariography

Multple deep trabeculations into the myocardium


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TREATMENT

Standard therapy for Heart Failure

Cardiac Resynchronization Therapy

Insertion of Implantalbe CardioverterDefibrillator

Cardiac Transplantation


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HYPERTROPHIC

CARDIOMYOPATHY


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LV hypertrophy

Two Features:

Asymmetric LV hypertrophy

Dynamic LV outflow tract pressure gradient

Diastolic Dysfunction ubiquitous

pathophysiologic abnormality Distinctive pattern of hypertrophy


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CLINICAL FEATURES

First Clinical Manifestation: Sudden Cardiac

Death

Dyspnea most common complaint

Syncope

Angina pectoris

Fatigue


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PHYSICAL EXAMINATION

Double or triple apical precordial impulse

Fourth heart sound

Systolic Murmur

Hallmark of obstructive HCM

Typically harsh, diamond-shaped and begins well

after the first heart sound.


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HEMODYNAMICS

Pressure gradient is dynamic

Obstruction is due to narrowing of LV outflow

tract by systolic anterior movement of the

mitral valve against the hypertrophied septum

Three basic mechanisms:

Increased LV contractility

Decreased ventricular preload

Decreased aortic impedance and pressure


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LABORATORY EXAMINATIONS

ECG: LV hypertrophy and widespread, deep

broad Q waves.

Echocardiogram

Mainstay of diagnosis

LV hypertrophy, septum


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PROGNOSIS

SCD is major cause of mortality

Atrial Fibrillation common late in the disease

Infective Endocarditis


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INHERITED METABOLIC CM with LV

HYPERTROPHY

Cardiac Danon Disease

Glycogen Storage Cardiomyopathy

Fabry Disease

Friedreichs Ataxia


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TREATMENT

B- adrenergic blockers

Amiodarone

Nindihydropyridine calcium channel blockers

(verapmil and diltiazem)

Surgical Myotomomy/ Myectomy

Insertion of ICD


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RESTRICTIVE CARDIOMYOPATHY


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Abnormal diastolic function

Ventricular walls are excessively rigid and

impede ventricular filling

Partial obliteration of the ventricular cavity by

fibrous tissue and thrombus contributes to the

abnormally increased resistance

CLINICAL FEATURES


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CLINICAL FEATURES

Exercise intolerance

Dyspnea

Dependent edema

Ascites

Enlarged tender and often pulsatile liver

Elevated jugular venous pressure and does not

fall normally with inspiration (Kussmauls sign)

3rd and 4th heart sounds

Apex impulse easily palpable

Mitral regurgitation is common


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LABORATORY EXAMINATION

ECG:Low voltage, nonspecific ST-T wave abnormalitiesand various arrhythmias

Echocardiography, CTI and CMRI: symmetrically

thickened LV walls and normal or slightly reducedventricular volumes and systolic function; Atria usuallydilated

Doppler:Diastolic Dysfunction

Cardiac Catherterization: reduced cardiac output,elevation of LV and RV end-diastolic pressures, and a dip-and-plateau configuration


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ETIOLOGIES

Eosinophilic Endomyocardial Disease

Cardiac Amyloidosis


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EOSINOPHILIC ENDOMYOCARDIAL

DISEASE

Loefflers endocarditis or Fibroplastic Endocarditis

Occurs in temperate climates

Endocardium of either or both ventriles is thickened

Mitral regurgitation present on Doppler Large mural thrombi may develop in either ventricle

Hepatosplenomegaly and localized infiltration of otherorgans are present

Management: Diuretics, afterload-reducing agnets andanticoagulation


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CARDIAC AMYLOIDOSIS

Four clinical presentations:

Diastolic dysfunction

Systolic dysfunction

Arrhythmias and conduction disturbances

Orthostatic hypotension

2d-Echo: show thickened myocardial wall with a

diffuse, hyperrefractile speckled appearance Management: Chemotherapy, Heart

transplantation


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Other RCM

Iron-overload cardiomyopathy

(hemochromatosis)

Myocardial Sarcoidosis

Carcinoid Syndrome


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TREATMENT

Salt restriction

Diuretics

Digitalis impaired systolic function

Anticoagulation eosinophilic

endomyocarditis


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OT Management

Energy Conservation Techniques

Relaxation Techniques

Patient Education and Supportive Therapy

mf3 - cardiomyopathy

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