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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