CARDIOMYOPATHY

Cardiomyopathy (CMP)is 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.

( K.V. Krishnadas)

CARDIOMYOPATHY

A heterogeneous group of diseases of the

myocardium associated with mechanical

and/or electrical dysfunction, which usually

(but not invariably) exhibit inappropriate

ventricular hypertrophy or dilatation, and are

due to a variety of etiologies that frequently are

genetic.

( Hurst’s text book of cardiology)

RELATED ANATOMY AND

PHYSIOLOGY

RELATED ANATOMY AND

PHYSIOLOGY

• Cardiac muscle (heart muscle)

involuntary striated muscle

• The myocardium is the muscle tissue of the heart,

and forms a thick middle layer between the

outer epicardium layer and the

inner endocardium layer

INCIDENCE

• Dilated cardiomyopathy, the most common form,

affects five in 100,000 adults and 0.57 in 100,000

children.

• Hypertrophic cardiomyopathy, the leading cause of

sudden death in athletes, with an incidence of one in

500 persons.

• Restrictive cardiomyopathy and arrhythmogenic right

ventricular cardiomyopathy are rare, and their

diagnoses require a high index of suspicion.

CLASSIFICATION

OF

CARDIOMYOPATHIES

WHO classification of cardiomyopathy(1995)

American Heart Association

classification of cardiomyopathy

Primary

secondary

Secondary cardiomyopathy

• Infiltrative

• Storage

• Toxicity

• Inflammatory

• Endocrine

• Nutritional deficiencies

• Consequence of cancer therapy

• Autoimmune/ collagen

Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy

• It is characterized by inappropriate left

ventricular hypertrophy, decreased cardiac

output and outflow obstruction.

• Hypertrophic subaortic stenosis

Common causes

• Genetic(autosomal dominant) or familial

• Hypertension

• Ischemia( coronary artery disease)

• Aortic stenosis

PATHOPHYSIOLOGY

Four main characteristics

• Massive ventricular hypertrophy

• Rapid, forceful contraction of the left

ventricle.

• Impaired relaxation( diastole)

• Obstruction to aortic outflow

CLINICAL MANIFESTATIONS

• Rapid, pounding heartbeat.

• Chest tightness or pressure.

• Fluid retention resulting in swollen feet or

ankles or unexplained weight gain.

Diagnostic studies

• History and physical examination

• Palpation and auscultation of the chest.

• ECG findings

• Echocardiogram

• Chest X ray

• Cardiac catheterization

Management of hypertrophic

cardiomyopathy

GOAL

• Improve ventricular filling.

• Reduce ventricular contractility

• Relieve left ventricular outflow

obstruction.

Management of cardiomyopathy

Lifestyle changes

• Reduced alcohol consumption, weight loss,

exercise, smoking cessation, and a low-sodium

diet.

Management of cardiomyopathy

• ᵦ adrenergic blockers

• Calcium channel blockers

• Antidysrrhythmics

• Implantable Cardioverter Defibrillator

• Dual-Chamber Pacing

Symptomatic management

Percutaneous Transluminal Septal Myocardial

Ablation(PTSMA)

Surgical management

• Ventriculomyotomy and myectomy

Arrhythmogenic Right Ventricular

Cardiomyopathy/ Dysplasia

• It predominantly involves the right ventricle

with progressive loss of myocytes and

fibrofatty tissue replacement, resulting in

regional (segmental) or global

abnormalities.

• Genetic defects of the part of the heart

muscle, desmosome.

• It shows autosomal dominant inheritance

• 80% of individuals present with syncope,

palpitations or sudden cardiac death.

• Noninvasive tests

• 12-lead ECG, signal-average ECG,

echocardiography, right ventricular angiography,

CMR imaging, CT, and electroanatomic mapping

of the right ventricle

• Endomyocardial biopsy

Treatment

• includes lifestyle alterations (i.e., avoiding

intense physical activity), antiarrhythmic drugs,

and implantable cardioverter-defibrillators in

high-risk patients.

• Cardiac transplantation

ION CHANNELOPATHIES

• Brugada syndrome

• Long QT syndrome

• Short QT syndrome

• SUNDS( Sudden Unexplained Nocturnal Death

Syndrome)

• Idiopathic ventricular fibrillation.

Brugada Syndrome

• Associated with mutations in

the gene (SCN5A)that encodes for

the sodium ion channel in

the cellmembranes of the muscle cells of the

heart (the myocytes).

• A distinctive ECG pattern consisting of

right bundle-branch block and coved ST-

segment elevation in the anterior precordial

leads (V1–V3).

Management

• Treatment of underlying arrhythmias

• Implantable cardioverter defibrillator

• Quinidine class Ia antiarrhythmic.

DILATED CARDIOMYOPATHY

DILATED CARDIOMYOPATHY

It is characterized by dilatation of the

ventricles with subsequent impairment of

systolic function.

Most common cardiomyopathy

ETIOLOGY

• Idiopathic

• Autosomal dominant , recessive and X-

linked modes of inheritance.

• Gene mutation

• Inflammatory and Infectious Myocarditis

• Autoimmunity

OTHER CAUSES

• Cardiotoxic agents- alcohol, cocaine,

doxorubicin( adriamycin)

• Hypertension

• Ischemia ( coronary artery disease)

• Metabolic disorders

• Muscular dystrophy

• Pregnancy

• Valve disease

PATHOPHYSIOLOGY

Diffuse inflammation and rapid degeneration of myocardial fibers.

Ventricular dilation Cardiomegaly

Impairment of systolic function(contractile dysfunction)

Atrial enlargement and stasis of blood in the left ventricle.

Heart becomes weak and the chambers get

large.

Heart cannot pump enough blood out to the

body

Decreased cardiac output

• Chamber enlargement frequently leads to a

dilation of the valvular orifice.

• Intracavitary thrombi located in the

ventricular apices

CLINICAL MANIFESTATIONS

• Decreased exercise capacity

• Fatigue

• Dyspnea at rest

• Paroxysmal nocturnal dyspnea

• Orthopnea

As the disease progresses the patient may

experience

• Dry cough

• Palpitations

• Abdominal bloating

• Nausea

• Vomiting

• Anorexia

Signs

• Irregular heart rate with an abnormal S3

and/or S4

• Tachycardia or bradycardia

• Pulmonary crackles

• Edema

• Weak peripheral pulses

• Pallor

• Hepatomegaly

• Jugular venous distension

• Heart murmurs

• Dysrhythmias

DIAGNOSTIC STUDIES

• Doppler echocardiography

• Chest X-Ray

• ECG

• Elevated serum BNP( if heart failure)

• Cardiac catheterization

EMB (Endomyocardial biopsy)

• Biochemical testing

• Endocrine function

• Radionuclide imaging (radionuclide

ventriculography)

• Cardiac MRI

• Multidetector computed tomography

MANAGEMENT

Goal

• Enhance myocardial contractility

• Decrease afterload.

MANAGEMENT

• Nitrates (eg: Nitroglycerin)

• ACE inhibitors( Eg: captopril)

• ᵦ adrenergic blockers( Eg: metoprolol)

• Aldosterone antagonists ( Eg: spironolactone)

• Diuretics to maintain the volume balance.

• Cardiac glycosides( Eg: Digoxin)

MANAGEMENT

• Antiarrhythmics( Eg: Amiodarone)

• Anticoagulation therapy

• Treatement of underlying disease process.

• Continuous infusion of dobutamine followed

by aggressive diuresis.

• Implantable cardiac defibrillators.

• Biventricular pacemakers.

SURGERY

• Left ventricular reconstruction

• Implantation of external restraint devices

• Left ventricular assist devices

• Heart transplantation

EMERGING SPECIFIC THERAPIES

• Agents to eradicate persistent viral infections

and immunomodulatory agents.

• Stem cells for cardiac regeneration and gene

therapy approaches are in clinical trials.

RESTRICTIVE CARDIOMYOPATHY

Restrictive cardiomyopathy

• It is a disease of the heart muscle that impairs

diastolic filling and stretch.

• Systolic function remains unaffected.

• Least common of the cardiomyopathic

conditions.

• The heart chambers are unable to fill with

blood because the heart muscle is stiff.

ETIOLOGY

• Amyloidosis

• Scarring of the heart from an unknown cause.

• Myocardial fibrosis

• Hypertrophy and infiltration

Secondary causes includes

• Endomyocardial fibrosis

• Sarcoidosis

• Neoplastic tumor

• Ventricular thrombus

• Fibrosis of different etiology

• Radiation to the thorax

PATHOPHYSIOLOGY

Increase in stiffness of the ventricular walls

Impaired diastolic filling of the ventricle

Reducing preload and end-diastolic volume

Heart failure

As the disease progresses

Systolic dysfunction

CLINICAL MANIFESTATIONS

Classic symptoms of restrictive CMP are

• Fatigue

• Exercise intolerance

• Dyspnea

Additional symptoms

• Angina

• Orthopnea

• Syncope

• Palpitations

SIGNS OF HEART FAILURE

• Dyspnea

• Peripheral edema

• Ascites

• Hepatomegaly

• Jugular venous distension.

• Kussmaul sign

DIAGNOSTIC STUDIES

• Chest x ray

• ECG

• Echocardiography

• Endomyocardial biopsy

MANAGEMENT

GOAL

Improve diastolic filling

Treatment of underlying disease process

• Treatment include conventional therapy for

heart failure and dysrhythmias.

• Diuretics may help relieve symptoms

• Calcium channel blockers.

• Cardiac transplantation

COMPLICATIONS

Embolus formation.

Decreased ejection fraction allow stasis of blood

to occur in Lt ventricle.

Thrombus may lodged in spleen kidney,

extremities, cerebral or coronary circulation.

Dysarrhythmias.

Sudden cardiac death.

Stress provoked (Tako-tsubo or Broken

Heart Syndrome)

• An acute cardiomyopathy can be provoked

by a stressful or emotional situation or

exposure to high doses of catecholamines

(sympathomimetic drugs).

Typical presentation

• sudden onset of congestive heart failure

• ECG changes mimicking a myocardial infarction of the anterior wall.

• Bulging out of the left ventricular apex with a hypercontractile base of the left ventricle is often noted.

• "tako tsubo", or octopus pot in Japan.

Treatment

• Supportive management

• Intra-aortic balloon pump

• Fluids, and negative inotropes such as beta

blockers or calcium channel blocker

• Aspirin

• Common among middle-aged women

• In most cases is fully reversible with supportive care.

• ECG fingings- myocardial infarction in the presence

of left ventricular dysfunction and absence of

epicardial coronary stenoses should prompt the

diagnosis.

• Endomyocardial biopsy is of value to exclude

myocarditis.

Peripartum Cardiomyopathy

• Peripartum cardiomyopathy is defined as a

cardiomyopathy manifesting between the

last month of pregnancy and 6 months post

partum

• Orthopnea

• Dyspnea

• pitting Edema

• cough, frequent night-time urination,

• excessive weight gain during the last month of

pregnancy (1-2+ kg/week; two to four or more

pounds per week),

• Palpitations and chest pain.

COMPLICATIONS

• stroke, loss of circulation to a limb,

even coronary artery occlusion (blockage)

with typical myocardial infarction

Treatment

• similar to treatment for congestive heart

failure

NURSING MANAGEMENT

NURSING MANAGEMENT

Nursing Assessment

• Evaluate patient's chief complaint, which may include

fever, syncope, general aches, fatigue, palpitations,

dyspnea.

• Evaluate etiologic factors, such as alcohol abuse,

pregnancy, recent infection, or history of endocrine

disorders.

• Assess for positive family history.

• Auscultate lung sounds for crackles (pulmonary

edema) or decreased sounds (pleural effusion)

• Assess heart size through palpation of chest for point

of maximal impulse (PMI), and auscultate for

abnormal sounds.

• Evaluate cardiac rhythm and ECG for evidence of

atrial or ventricular enlargement and infarction.

Nursing Diagnoses

• Decreased Cardiac Output related to decreased

ventricular function and/or dysrhythmias

• Activity intolerance related to low cardiac output

• Fluid volume excess in related to ventricular

dysfunction

• Anxiety related to fear of death and hospitalization

• Fatigue related to disease process