Cardiomyopathy

Introduction
Cardiomyopathy is a dysfunction of the myocardium. It
is not associated with a structural deformity of the
heart.
The dysfunction can be secondary to such generalized
diseases as hypertension, amyloidosis,
hemochromatosis, SLE, leukemia, muscular dystrophy,
glycogenosis or gargoylism. However, the term is, by
convention, restricted to primary myocardial
involvement without any known cause
 Clinical Categories

• Congestive, characterized by CCF, arrhythmias,

murmurs of mitral and tricuspid incompetence
and emboli.
• Restrictive, characterized by restriction to
ventricular filling
• Obstructive, characterized by obstruction to
ventricular outflow.
 Congestive (Dilated)cardiomyopathy:

Dilated cardiomyopathy (DCM) refers to congestive

cardiac failure due to dilation and systolic dysfunction
of the ventricles (predominantly the left ventricle). It is
the most common form of heart muscle disease in
children.
 Contd.
DCM is reportedly more common in boys than in girls,
and some forms are clearly X-linked. All age groups are
affected. However, studies suggest that DCM is more
common in infants (age < 1 y) than in children. Fetal
presentation is uncommon.
Various factors have been identified as causes of
myocardial damage. However, in the vast majority of
patients, no specific etiology is demonstrable (ie,
idiopathic DCM).
Three major factors have been implicated in the
pathogenesis of myocardial damage in DCM: preceding
viral myocarditis, autoimmunity, and underlying genetic
predisposition.
Pathophysiology
• Injury to the myocardial cell is the initiating factor that
leads to cell death.
• If considerable cell loss occurs, the myocardium fails to
generate enough contractile force to produce adequate
cardiac output.
• This results in the activation of the following
compensatory mechanisms:
 The renin-angiotensin-aldosterone system
 Sympathetic stimulation
 Antidiuretic hormone production
 Release of atrial natriuretic peptide
• These compensatory mechanisms help to maintain
cardiac output in the initial phase; however, as myocardial
damage progresses, persistent and excessive activation
can be detrimental to cardiac function, leading to overt
congestive heart failure.
 Contd..
• Over-stretching of the ventricles causes myocardial
thinning, cavity dilation, secondary valvular
regurgitation, and compromised myocardial perfusion.
• Myocardial remodeling is an important contributor to
worsening heart failure. [4] Lost myocyte cells are
replaced with fibrous tissue, thereby decreasing the
compliance of one or more ventricles and adversely
affecting performance.
• Heightened peripheral vasoconstriction, abnormal and
excessive remodeling of the peripheral vasculature,
and abnormalities in endothelium-dependent
vasodilation contribute to the progression of heart
failure.
 Signs and symptoms

Onset of DCM is usually insidious but may be acute in as many at

25% of patients. Approximately 50% of patients with DCM have a
history of preceding viral illness. Initial presenting symptoms
typically include the following:
 Cough , Poor feeding , Irritability , Shortness of breath ,
Pallor , Sweating , Fatigability , Failure to gain weight,
Decreased urine output . Wheezing may be an important
clinical sign, suggesting congestive heart failure in infants.
Other symptoms at presentation, found in approximately 20% of
patients, are as follows:
 Chest pain, Palpitations, Orthopnea, Hemoptysis, Frothy
sputum, Abdominal pain, Syncope, Neurologic deficit
 Diagnosis

• Echocardiography and Doppler studies for diagnosing the type of

cardiomyopathy and the degree of dysfunction in the heart muscle.
• Chest radiography can reveal cardiomegaly and pulmonary edema.
• ECG can evaluate the amount of cardiomegaly and reveal any
abnormal heart rhythm.
• The complete blood count, erythrocyte sedimentation rate, and C-
reactive protein level may show evidence of acute inflammation in
patients with DCM in the presence of active myocarditis.
• Cardiac catheterization should be performed only when absolutely
essential. Children with DCM are at a particular risk for complications
during cardiac catheterization studies and angiography.
• Myocardial biopsy is usually performed in preparation for cardiac
transplant and post-transplant follow-up monitoring.
 Management

• Diuretics, angiotensin-converting enzyme (ACE)

inhibitors, and beta-blockers are used.
• General supportive measures including endotracheal
intubation and mechanical ventilation, vasoactive
infusions, and fluid/acid-base management.
• Treat chest infections appropriately.
• Treat anemia appropriately.
• Supplemental oxygen is of benefit only in patients with
hypoxia (as with pneumonia or pulmonary edema).
• Cardiac transplantation is currently the optimal
treatment for DCM-induced resistant chronic heart
failure in children. Survival rates of as much as 92% at 5
years and 53% at 15 years have been reported.
 Restrictive cardiomyopathy
• Restrictive cardiomyopathy (RCM) is a rare disorder
in children that is characterized by restrictive filling
and reduced diastolic volume of one or both
ventricles with normal or near-normal systolic
function and wall thickness. The heart is structurally
normal, although histologic abnormalities are often
present, depending on the etiology of the restrictive
cardiomyopathy.
• Idiopathic RCM has been described in children of all
ages. It may be slightly more common in girls than in
boys.
• In most pediatric cases of RCM, the etiology is
unknown. Risk factors are also unknown.
 Pathophysiology
The pathophysiology of RCM is diverse. This condition can
be associated with diseases such as
amyloidosis, hemosiderosis, hypereosinophilia,
and endocardial fibroelastosis; it can also occur secondary
to radiation therapy and certain medications. However,
these may be considered separate diseases because the
etiology is known.
Morphologic findings include atrial enlargement without
increased ventricular wall thickness or ventricular cavity
dilation.
 Signs and symptoms
• The patient with restrictive cardiomyopathy is
generally in CCF with dependent edema, ascites
and an enlarged, tender liver.
• The JVP is persistently elevated.
• Cardiomegaly is invariably present.
• The heart sounds are distant . Usually, third and
fourth heart sounds are audible.
• Murmurs are, as a rule, not distinctive.
 Diagnosis
• The main differential diagnosis for restrictive cardiomyopathy
(RCM) is constrictive pericarditis. Differentiating the 2
conditions can be difficult, particularly in children .
• Cardiac catheterization and Doppler echocardiography are
generally able to distinguish between RCM and CP, although
both are characterized by abnormal diastolic ventricular filling
and elevated ventricular end-diastolic pressures.
• Cardiac magnetic resonance imaging may also be helpful if
stigmata of constrictive pericardial disease are present.
• X-ray chest shows cardiomegaly without any
calcification in the region of pericardium.
• ECG shows arrhythmias and ST-T wave changes are common.
 Management
• Therapy is limited to symptomatic treatment and is often
ineffective in improving outcome.
• Diuretics reduce pulmonary or systemic venous congestion,
anticoagulation should be considered because of the
significant risk of thromboembolic complications.
• Surgical options in children with RCM are limited to heart
transplantation. The results of heart transplantation are
generally quite good but depend on the degree of pulmonary
hypertension and resultant postoperative complications.
• A normal diet is recommended.
• The degree of hemodynamic abnormality and the risk of
sudden death are significant, and patients should be
restricted from competitive athletics, despite a paucity of
data on the subject.
Obstructive(hypertrophic) cardiomyopathy

• Hypertrophic cardiomyopathy is a primary

genetic myocardial disease usually
characterized by asymmetric ventricular septal
hypertrophy. It is characterized by
hypertrophy of the left ventricle which
sometimes involves the right ventricle. The
disease course is highly variable, but it is well
recognized that there is an increased risk of
morbidity and sudden cardiac death .
 Epidemiology
• HCM does not have a racial or ethnic
predisposition and has been reported in patients
of all races.
• This condition may occur at any age, from the
newborn to the elderly. Overall, its most common
presentation is in the third decade of life. Among
children younger than 18 years diagnosed with
HCM, the median age at diagnosis is 7 years; one
third are diagnosed before age 1 year.
 Signs and symptoms
• The signs and symptoms of HCM in pediatric
patients vary based on age and associated
conditions. Presenting symptoms may include:
• Chest pain
• Presyncope/syncope
• Palpitations
• Heart failure symptoms (eg, poor feeding,
failure to thrive, tachypnea, easy fatigability)
• Sudden cardiac arrest/death
The signs and symptoms of HCM vary somewhat according to age:
Infants <1 year – An isolated heart murmur is the most common
presentation during the first year of life
• Children ≥1 year of age – Most older children (≥1 year of age)
with HCM are asymptomatic; however, children with inborn
errors of metabolism have a higher rate of presenting with
symptomatic heart failure. Among those who come to clinical
attention, symptoms may include:
• Abdominal pain, decrease in appetite, or intolerance of feeds
• Dyspnea on exertion
• Fatigue
• Atypical or anginal chest pain
• Presyncope and syncope, particularly during or immediately
following exertion
• Palpitations
• Sudden cardiac arrest/death
 DIAGNOSTIC EVALUATION

• Common electrocardiographic (ECG) findings

in individuals with hypertrophic
cardiomyopathy (HCM) include ST-T wave
abnormalities and left ventricular hypertrophy
Echocardiography
LV hypertrophy, Systolic anterior motion of the
mitral valve, LVOT obstruction.
Genetic testing
Additional testing
• Ambulatory ECG monitoring- should be
performed for 24 to 48 hours in all pediatric
patients diagnosed with HCM (based on
clinical and imaging findings) as part of the
risk assessment for ventricular arrhythmias
and risk for sudden cardiac death.
• Exercise testing - exercise on a treadmill usually
combined with echocardiography
• Cardiac magnetic resonance imaging
• Cardiac catheterization and biopsy - The
hallmark finding is increased LV wall thickening,
without an identifiable hemodynamic cause (eg,
hypertension, valve disease). Endomyocardial
biopsy is not regularly used for diagnosis but
may be useful to exclude non-sarcomeric
disease
 Management
Medical management of hypertrophic cardiomyopathy
(HCM) in children should focus on the following:
• Ruling out secondary causes
• Following for progression of disease and identifying
those with obstruction
• Controlling symptoms and restricting activity (with
avoidance of volume depletion)
• Identifying those at risk for sudden cardiac death
• Screening family members
 Cont,d.
Subacute bacterial endocarditis prophylaxis is not required.
Beta-blockers and calcium channel blockers (eg, verapamil
or diltiazem) are used to treat children with hypertrophic
cardiomyopathy (HCM). In individuals with significant
tachyarrhythmias, amiodarone and other class III-type
antiarrhythmic agents have also been used.
Avoid administration of the following agents:
• Inotropic drugs
• Nitrates and sympathomimetic amines, except in patients
with HCM and concomitant coronary artery disease
• Digitalis, because glycosides are contraindicated, except
in patients with uncontrolled atrial fibrillation
• Diuretics, because of their effect on left ventricular
volume
 Cont,d.
• Left Ventricular Myectomy
• Pacemaker Implantation
• Catheter Septal Ablation
• Implantable Cardioverter Defibrillator
The implantable cardioverter defibrillator (ICD) has
been used for prevention of sudden arrhythmic
death. Transvenous placement is similar in
technique to permanent pacemaker implantation.
 Nursing management
Nursing diagnosis
• Decreased Cardiac Output related to
impaired ventricular filling, contractility, or
outflow obstruction.
Plan & Goals :Client will maintain BP within
specific limits.
Implementation Rationale

Monitor Vital Signs & Decreased cardiac output stimulates SNS to increase HR to compensate
Oxygen saturation. for decreased C.O. Diastolic may be high as well as tachycardia present
initially, but in late stages everything falls.
Monitor BNP. BNP levels indicate the level of heart failure: As cardiac index decreases
& left ventricular pressure increase, BNP levels increase

Auscultate heart & S1 & S2 may be diminished if cardiac function is poor. S3 gallop present
breath sounds in early stage HF. S4 gallop may also be present. Crackles are often
regularly. heard @ bases of lungs, dyspnea & shortness of breath indicate HF
worsening=late stages.
Administer oxygen. Improves oxygenation of the blood, decreasing the effects of hypoxia &
ischemia.

Administer prescribed Administering medications are used to decrease the cardiac workload
medications. & increase effectiveness of contractions.

Encourage rest & Rest will decrease stress on the heart & decrease cardiac workload.
elevate head of bed. Elevating head of bed reduces work of breathing.Provide bedside
commode & assist with ADL's.
NURSING DIAGNOSIS:
• Fluid Volume Excess Related to Decreased
Cardiac Output
Plan & goals :
• Patient will modify diet to support long-term
management of condition
 Implementation Rationale
Assess respiratory status & auscultate Declining respiratory status signifies worsening left-
lung sounds @ least Q 4 hours. sided HF.

Monitor Intake & Output, weigh daily, & A decrease in urine output may indicate decrease in
notify physician if output is less cardiac output & renal ischemia. Weighing is an
objective measurement for fluid status

Record abdominal girth Q shift. Note Venous congestion can lead to ascites & may affect
complaints of : loss of appetite, GI function & nutritional status.
abdominal discomfort, or nausea.

Monitor hemodynamic labs. Provides a means of monitoring condition &

response to treatment.

Restrict fluids as ordered. Allow choices Providing choices increases patient's sense of
of fluid & timing of intake. Schedule control. Ice chips, hard candies, & frequent mouth
during am & afternoon. Offer ice chips & care relieve dry mouth & thirst & promote comfort.
frequent mouth care.
Nursing Diagnosis:
• Activity Intolerance Related to Decreasing
Cardiac Function
Plan & expected outcomes:
Patient will alter lifestyle to demonstrate
adjustment to alterations in activity level caused
by disease process.
Implementation
Organize nursing care to allow for rest
periods.
Assist with ADL's as needed & encourage
independence within limits.
Plan & implement progressive activities.
Uses passive & active R.O.M.
Rationale
Grouping care activities together allows
for adequate time to recharge
Ensures that care needs are met while
reducing cardiac workload. Involving
patient promotes a sense of control &
reduces sense of helplessness.
Progressive activities slowly increases
exercise capacity by strengthening &
improving cardiac function without strain.
Activity also helps prevent skeletal muscle
atrophy. ROM activities helps prevent
complications in severely compromised
patients.