CARDIOMYOPATHY

Presented By-
Joanna Rachel P M,
M Sc N 1ST YEAR,
Acon, Ch-95.
REVIEW OF ANATOMY & PHYSIOLOGY
 INTRODUCTION

Cardiomyopathies are a mixed group of diseases of

the myocardium (cardiac muscle) defined by
structural or functional abnormalities that negatively
affect the pump function of the heart.
In some types, there is obstruction to the outflow of
blood during the cardiac cycle.
 Cont.

Advances in clinical and genomic medicine have led

to better detection and understanding of
cardiomyopathies (some of which have a strong
genetic component), while high-profile cases of
sudden cardiac death have increased awareness of
the conditions.
 DEFINITION

“A heterogeneous group of diseases of the

myocardium associated with mechanical and/or
electric dysfunction that usually exhibit inappropriate
ventricular hypertrophy or dilatation due to a variety of
etiologies that are frequently genetic”.
 CLASSIFICATION

Cardiomyopathies were previously divided into

primary and secondary:
 primary cardiomyopathy was confined to the cardiac
muscle
secondary cardiomyopathy was part of a systemic
disorder affecting multiple organs.
 Cont.

cardiomyopathies according to morphological and

functional phenotype

Dilated
Hypertrophic
Restrictive
Arrhythmogenic
Unclassified
 INCIDENCE

 Dilated cardiomyopathy is the most common form,

affects 5 in 100,000 adults.
 Hypertrophic cardiomyopathy the leading cause of
sudden deaths in athletes with incidence of 1 in 500
people.
 Restrictive and arrhythymogenic cardiomyopathy
are rare.
 DILATED CARDIOMYOPATHY (DCM)

 Dilated cardiomyopathy is a condition in which the

heart becomes weak and the chambers get large.
 As a result, the heart cannot pump enough blood
out to the body.
 Dilation (enlargement) of all chambers (both atria
and both ventricles).
 The total size of the heart is typically huge
(cardiomegaly).
 HYPERTROPIC CARDIOMYOPATHY (HCM)

 Hypertrophic cardiomyopathy is a condition in which

the heart muscle becomes thick.
 It is a genetically inherited disease.
 The classic anatomic feature is the profound
hypertrophy of the myocardium of the left ventricle.
 Cont.

 This extra-thickened interventricular septum is

referred to as asymmetric septal hypertrophy (ASH).
 The IVS can become so hypertrophied that it bulges
into the lumen of the LV, thereby decreasing the
volume of the LV chamber.
 RESTRICTIVE CARDIOMYOPATHY (RCM)

 Restrictive cardiomyopathy is a myocardial disorder

that usually results from increased myocardial
stiffness that leads to impaired ventricular filling.
 Biventricular chamber size and systolic function are
usually normal or near-normal until later stages of
the disease.
 Affecting either or both ventricles, RCM may cause
signs or symptoms of left or right heart failure.
 Cont.

 Arrhythmias and conduction disturbances are frequently

encountered
 ARRHYTHMOGENIC CARDIOMYOPATHY
(ACM)
 Arrhythmogenic cardiomyopathy (AC) is a heart
muscle disease clinically characterized by life-
threatening ventricular arrhythmias and
pathologically by an acquired and progressive
dystrophy of the ventricular myocardium with fibro-
fatty replacement.
 Cont.

 The condition is a significant cause of sudden

cardiac death due to electric instability and
subsequent ventricular tachycardia or ventricular
fibrillation.
 UNCLASSIFIED CARDIOMYOPATHY

Peripartum cardiomyopathy
 Peripartum cardiomyopathy is a rare life threatening
condition that occurs around the last month of
pregnancy and up to six months postpartum.
 It has similar clinical characteristics to dilated
cardiomyopathy (DCM) such as ventricle dilation
and systolic dysfunction
 Cont.

Stress-induced cardiomyopathy
 Stress-induced cardiomyopathy (or takotsubo
cardiomyopathy) is often preceded by intense
emotional or physical stress, with likely
catecholamine release including adrenaline and
noradrenaline.
 Oestrogen deficiency in postmenopausal women
has also been implicated
 Cont.

Left ventricular non-compaction

 Left ventricular non-compaction (or spongy
myocardium) is a rare congenital cardiomyopathy.
 It typically affects the apex of the heart and is
defined by an altered myocardial wall with prominent
trabeculae and deep intertrabecular recesses, which
results in a thickened myocardium with two layers.
 Cont.

Histiocytoid cardiomyopathy
 Histiocytoid cardiomyopathy (or Purkinje cell
hamartoma) is a rare cardiomyopathy that presents
between birth and the age of four years and is more
common in females.
 It is associated with arrhythmias and sudden cardiac
death as well as congenital heart defects.
 Cont.

 It may occur in conjunction with extra-cardiac

features, including abnormalities of the nervous
system and the eyes.
 RISK FACTORS

 Family history.

 Coronary heart disease.

 Diabetes.

 Long term high blood pressure.

 Severe Obesity.
 Excessive alcohol consumption.
 CAUSES
 Dilated Cardiomyopathy

Genetics.
Infectious myocarditis.

Hypertension.
Heart valve disorders.
Myocardial infraction.

Chronic alcohol/cocaine use.

Neuromuscular disorders.
 Cont.

 Hypertrophic cardiomyopathy

Genetic factors.

Hereditary disorders.

 Restrictive cardiomyopathy

Amyloidosis.

 Radiation to the thorax.

 Endocardial fibrosis.
 PATHOPHYSIOLOGY

Idiopathic aetiology

Various myocardial insults

Decreased amount of cardiomyocytes

Dilatation of ventricles, decreased contractility of

myocardium, thrombosis and emboli
Systolic and diastolic dysfunction, hemodynamic
dysfunction
Heart insufficiency
 Cont.
Dilated cardiomyopathy

Hypertrophic Left ventricular Restrictive

Cardiomyopathy dysfunction Cardiomyopathy

Blood stasis in heart chambers

Heart failure
 CLINICAL MANIFESTATIONS

 patient presents with signs and symptoms of heart

failure (e.g., dyspnea on exertion, fatigue).
 Patients also report paroxysmal nocturnal dyspnea,
cough (especially with exertion), and orthopnea
 Other symptoms include fluid retention, peripheral
edema, and nausea, which is caused by poor
perfusion of the gastrointestinal system.
 Cont.

 The patient may experience chest pain,

palpitations, dizziness, nausea, and syncope with
exertion.
 However, with HCM, cardiac arrest (ie, sudden
cardiac death) may be the initial manifestation in
young people, including athletes.
 DIAGNOSTIC STUDIES

 The echocardiogram is one of the most helpful

diagnostic tools because the structure and function
of the ventricles can be observed easily.
 ECG demonstrates dysrhythmias and changes
consistent with left ventricular hypertrophy.
 The chest x-ray film reveals heart enlargement and
possibly pulmonary congestion.
 Cont.

 Cardiac catheterization is sometimes used to rule

out coronary artery disease as a causative factor.
 An endomyocardial biopsy may be performed to
analyze myocardial tissue cells
 MANAGEMENT

 The overall goals for cardiomyopathy are to

manage your signs and symptoms, prevent your
condition from worsening, and reduce your risk of
complications.
 PHARMACOLOGICAL MANAGEMENT

 This involves treating the associated heart failure,

and arrhythmias with medications like beta blockers
and ACE inhibitors.
 Vasodilators, such as dobutamine, and milrinone,
which help decrease the heart’s workload.
 Diuretics to reduce fluid overload.
 Anticoagulants can also be administered to prevent
thrombus formation.
 NUTRITIONAL MANAGEMENT

 Restrict salt intake (sodium) to 2,000 to 3,000 mg

per day
 Avoid oily foods
 Initially Liquid diet is advised later progressing to
soft solid & semi-solid foods.
 EXECRISE

 Adequate rest will relieve chest pain & pressure

 Advice to do non-competitive daily activities
 Elevation of legs & Feet during sleep to improve
venous return to the heart
 Avoid strenuous Exercises, lifting Heavy Objects &
activities that leads to dehydration.
 INTERVENTIONAL THERAPY

Percutaneous trans-luminal Septal Myocardial

Ablation
 This procedure consists of administering alcohol into
the first septal artery branching off the left Anterior
descending artery, which causes Ischemia & septal
wall Myocardial Infarction.
 Ablation of the septal wall will decrease the outflow
obstruction & improves symptoms of Heart Failure.
 SURGICAL MANAGEMENT

Ventriculomyotomy: 
 It involves incising the hypertrophied  Septal
Muscles to relieve obstruction of the aortic outflow.
Septal Myectomy:
 It is a surgical procedure, where a small amount of
the thickened septal wall  is removed to widen the
outflow tract (the path the blood takes) from the left
ventricle to the aorta.
 Cont.

Cardiac Resynchronization Therapy (CRT):

 In some patients progressing to heart failure,
biventricular pacing (a pacemaker that senses and
initiates heartbeats in the right and left ventricle) is
applied to improve survival, reduce symptoms and
increases exercise  tolerance.
 Cont.

Implantable Cardioverter Defibrillator (ICD):

 ICDs are suggested for people at risk for life-
threatening arrhythmias or sudden cardiac death.
 The ICD is a small device placed just under the skin
and is connected to wire leads that are threaded
through the vein to the heart. 
 Cont.

 It constantly monitors the heart rhythm, when it

detects a very fast, abnormal heart rhythm, it
delivers energy to the heart muscles & reverts into
normal heart rhythm.
 Cont.

Ventricular Assist Devices (VAD):

 A left ventricular assist device (VAD — also called
ventricular assist system or VAS) is a type of
mechanical circulatory support device (MCSD),
where a mechanical pump is implanted in patients
who have progressed to heart failure. This device
assists the weakened left ventricle to pump &
maintain Systemic Circulation
 Cont.
Heart Transplantation: 
 In heart transplant, the donor heart will be placed in
proper position and attached to your major blood
vessels
 Orthotopic Transplant: The patient’s own heart is
removed and replaced with the donor heart 
 Heterotopic Transplant: The recipient’s (patient)
diseased heart is left in place to support the donor
heart
 NURSING MANAGEMENT

 Administer oxygen therapy as prescribed

 Monitor the client continuously for vital signs,

saturation, I/O chart, level of consciousness
dysrhythmias etc.
 Administer IV fluids as Prescribed
 Monitor the Nutritional Needs of the client by
Inserting NG feeding tube if oral feeds are not
tolerated
 Cont.

 Keep all emergency articles like suctioning, Endo-

tracheal Intubation tray, Defibrillators & Mechanical
Ventilator at the bed side to meet emergencies 
 Alleviate the symptoms & prevent complications
 Administer all medications & set the Infusion rate of
emergency drugs as Prescribed
 Cont.

 Provide Psychological & Emotional Support to

patient & family members
 Prepare the client for Invasive therapies & Surgical
Intervention, when necessary
 Administer Prophylactic Antibiotics for peri-operative
Client to prevent Infection
 Advice the client to keep the legs & feet elevated
with a pillow in bed to prevent & treat Edema
 Cont.

 Follow Anti- coagulant prophylaxis as prescribed or

use Anti- Embolic Stockings & Sequential
Compressive Devices as prescribed
 Teach & perform Hygienic Practices & meet the
activities of Daily living for the client
 Monitor the Skin regularly & prevent Bedsores by
using Alpha bed or air mattresses
 NURSING DIAGNOSIS
 Decreased cardiac output related to structural
disorders caused by cardiomyopathy or to
dysrhythmia from the disease process and medical
treatments.
 Ineffective cardiopulmonary, cerebral, peripheral,
and renal tissue perfusion related to decreased
peripheral blood flow (resulting from decreased
cardiac output).
 Cont.
 Impaired gas exchange related to pulmonary
congestion caused by myocardial failure (decreased
cardiac output)
 Activity intolerance related to decreased cardiac
output or excessive fluid volume, or both
 Anxiety related to the change in health status and in
role functioning
 Cont.

 Powerlessness related to disease process

 Noncompliance with medication and diet therapies

 PATIENT AND FAMILY EDUCATION

 Instruct patient to take all medicines as prescribed

and to follow with health care provider.
 Advice the client to take low sodium & low
Cholesterol diet.
 Encouraged to drink 6 to 8 glasses of water per day
unless restricted in case of Edema
 Advise the patient to avoid alcohol, smoking,
caffeine and over the counter drugs
 Cont.

 Advice the client to Schedule adequate periods of

rest & Activity
 Instruct the patient to avoid lifting of heavy weights
and vigorous activities but perform mild to moderate
Exercises & activities of Daily living
 Encourage the use of stress reduction activities like
relaxation, meditation, distraction & prayers to
relieve tension.
 Cont.
 Instruct the patient to report the hospital any signs of
heart failure like increased weight gain, edema,
shortness of breath and increased fatigue.
 Demonstrate CPR procedure to the family members
& care givers & advice them to perform CPR in case
of sudden cardiac arrest at home.
 Instruct the patient to notify the physician before any
dental and medical procedures to be done as there
is a high risk for Infective endocarditis.