CARDIOMYOPATHIES

Hypertrophic, Restrictive and Dilated

ROSE MARIE LEE

CARDIOMYOPATHIES
NAME: ROSE MARIE LEE

According to the National Library of Medicine, Cardiomyopathy is a weakening of the

heart muscle or another problem with the heart muscle. It often occurs when the heart
cannot pump as well as it should, or with other heart function problems. They are
attributed to intrinsic myocardial dysfunction. They can be primary or secondary. There
are three major relatively common types and these include: dilated cardiomyopathy,
hypertrophic cardiomyopathy and restrictive cardiomyopathy. There is also
arrythmogenic and unclassified but this paper will serve to research these types of heart
diseases.

DILATED CARDIOMYOPATHY:

This is characterized by progressive cardiac dilation and contractile (systolic

dysfunction), usually with concurrent hypertrophy. The patient will have signs of heart
failure including fatigue, Shortness of breath (dyspnea) when you're active or lying down,
reduced ability to exercise, Swelling (edema) in your legs, ankles and feet, and Swelling
of your abdomen (ascites). The myocardium will first be compromised and become thin
with diffuse dilation causing compensatory mechanisms to cause hypertrophy. Associated
with mitral or tricuspid regurgitation.

If the cause is ischemia only the left ventricle will be affected otherwise, both ventricles
are affected.
Atrial fibrillation occurs as the left atrium dilates and mural thrombi are frequently
formed when dilation is significant.

Etiology
There are known and unidentified causes. Known causes include CAD with diffuse
ischemic myopathy, chronic tachycardia, granulomatous diseases, viral infection
(coxsachie B, Trypanosoma cruzi), toxoplasmosis, thyrotoxicosis, nutritional diseases,
alcohol, drugs, toxins, tumors, connective tissue disorders, pregnancy, emotional stress
and genetics.

Signs and symptoms

This depends on which ventricle is affected.
Left ventricle:

Exertional dyspnea
Fatigue
Chest pain
Elevated LV diastolic pressure
Low cardiac output

Right ventricle:

Peripheral edema

Neck vein distention

Sudden death due to ventricular tachyarrythmias (common cause of sudden death

in young patients)
Chest pain

Prognosis
There is a poor prognosis where 50% of deaths are sudden due to arrhythmia or embolic
event. Prognosis is better if ventricular hypertrophy preserves wall thickness but worse if
wall thins a lot and the ventricle dilates

Treatment
Treat primary disease or use same treatment for heart failure. That includes: ACEinhibitors, ARBs, beta-blockers, aldosterone receptor blockers, angiotensin II receptor
blockers, diuretics, digoxin and nitrates.

Diagnosis
Chest Xray, ECG, Echocardiography, MRI, Coronary angiography

Medical Management

Lifestyle Restriction
Avoid alcohol because of cardiotoxic effects. There should be a 2-g salt
restriction, half- gallon- fluid restriction, daily endurance- type exercise for
about 30 minutes and daily weight recordings.

Drugs (see treatment)

Heart Transplant and left ventricular assist device
For advanced disease that is refractory to other therapies.

Cardiac resynchronization therapy and implantable defibrillators

Includes insertion of a implantable cardioverter defibrillator (ICD) for advanced
diseases, reverse remodeling and primary and secondary prevention.
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Valvular Replacement
Anticoagulants

(Merck Manual, 2014)

HYPERTROPHIC CARDIOMYOPATHY:
HCM is marked by ventricular hypertrophy and diastolic dysfunction without an
increased afterload (aortic stenosis, coarctation of aorta, systemic hypertension)
There are three prominent histologic markers of HCM:

Myocardial disarray in cellular architecture

Small vessel disease responsible for silent ischemia and
Scarring

This presents in the patient as electrical instability and ventricular arrhythmias. Exertional
dyspnea, exercise intolerance and fatigue reflect heart failure which can be associated
with chest pain with the absence of CAD. Symptoms are dues normally to:

Left ventricular outflow obstruction (elevated LV pressures and wall stress)

Diastolic dysfunction and impaired LV filling from non compliant thickened wall

and
Myocardial ischemia from small vessel disease.

The myocardium is abnormal with cellular and myofibrillar disarray, although this
finding is not specific for HCM.
In the most common form, the upper interventricular septum below the aortic valve is
markedly hypertrophied and thickened, with little or no hypertrophy of the left
ventricular (LV) posterior wall; this pattern is called asymmetric septal hypertrophy and
appears to accelerate during puberty. During systole, the septum thickens, and sometimes
the anterior leaflet of the mitral valve, already abnormally oriented because of the

abnormally shaped ventricle, is sucked toward the septum by a Venturi effect of high
velocity blood flow, further obstructing the outflow tract and decreasing cardiac output.
The resulting disorder may be termed hypertrophic obstructive cardiomyopathy. Less
commonly, mid-ventricular hypertrophy leads to an intracavitary gradient at the papillary
muscle level. In both forms, the distal LV may ultimately thin and dilate. Apical
hypertrophy can also occur but does not obstruct outflow, although it may obliterate the
apical portion of the LV during systole. Sometimes the hypertrophy is diffuse and
symmetrical.
Hypertrophy results in a stiff, noncompliant chamber (usually the LV) that resists
diastolic filling, elevating end-diastolic pressure and thus increasing pulmonary venous
pressure. As resistance to filling increases, cardiac output decreases, an effect worsened
by any outflow tract gradient present. Because tachycardia allows less time for filling,
symptoms tend to appear mainly during exercise or tachyarrhythmias.

Etiology
The higher percentage of incidence is familial. This is rarely acquired but may develop
in patients with cardiomegaly, pheochromocytoma and neurofibromatosis.

Signs and Symptoms

Patients that are 20-40 years show exertional signs, however:

dyspnea
chest pain
palpitations
syncope(marker of increased risk of sudden death) due to ventricular tachycardia

or fibrillation
no fatigue (systolic function preserved)
BP and heart rate normal
Sustained thrust at apex beat
S4 sound
Septal hypertrophy produces systolic ejection- type murmur at left sternal angle in
3rd or 4th intercostal space.

Diagnosis
Echocardiography, MRI, 2-D Doppler echocardiography can differentiate the forms of
cardiomyopathy.

Prognosis
Mortality rate is inversely proportional to age. Worse prognosis is seen for young patients
with family history of sudden death.

Treatment
Beta-blockers, calcium channel blockers (verapamil), anti-arrythmics

Medical Management
Major risk markers for primary and secondary prevention include:

Prior cardiac arrest

Sudden death due to HCM in one ore more relatives
Massive left ventricular hypertrophy >30mm
Non-sustained ventricular tachycardia
Hypotensive or attenuated bp during exercise
Unexplained syncope related to exertion

Drugs (see treatment)

Surgery
Septal myectomy

Implantable defribrillator

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Lifestyle RestrictionYoung athletes are encouraged not to participate in intense, competitive sports

Heart Transplant
Mitral valve replacement
Ablation of AV node

(Merck Manual, 2014)

RESTRICTIVE CARDIOMYOPATHY:

Restrictive cardiomyopathy is a rare form of heart disease that is characterized by

restrictive filling of the ventricles. In this disease the contractile function of the heart and
wall thickness are usually normal, but the relaxation of filling phase of the heart is very
abnormal. This occurs when the heart muscle is stiff and poorly compliant and does not
allow the ventricular chambers to fill with blood normally. This causes a backlog of blood
in the atria, lungs and body causing signs of heart failure.

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It is a condition characterised by normal left ventricular cavity size and systolic function
but with increased myocardial stiffness. This makes the ventricle incompliant and fill
predominantly in early diastole. It is often associated with raised left atrial pressure, atrial
dilatation and sometimes arrhythmias.

This is the least prevalent form of cardiomyopathy. There are two types:

Non- obliterative (myocardial infiltration by an abnormal substance)

Obliterative (fibrosis of the endocardium and subendocardium)

Etiology
Like other cardiomyopathies there are known and unknown causes. Known causes among
other disorders include:

Genetic abnormalities
Connective tissue disorders
Tumors
Radiation
Hypereosinophilic syndrome

Signs and Symptoms:

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Mimics those of constrictive pericarditis

Exertional dyspnea
Repeated lung infections in children
Orthopnea
Peripheral edema
Fatigue
Ventricular arrhythmias
Ascites
Hepatomegaly

Diagnosis

Echocardiography, MRI and testing for cause (eg. Rectal biopsy for amyloidosis, iron
tests or liver biopsy for hemochromatosis).

Prognosis

Prognosis of RCM is poor because diagnosis is often made at the late stage. No treatment
is available for most patients; symptomatic, supportive care can be provided.

Treatment

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Treat primary cause

Diuretics
Septal myectomy, pacemaker implantation, implantable cardioverter defibrillator.

The goal of treatment in RCM is to reduce symptoms by lowering elevated

filling pressures without significantly reducing cardiac output. Beta
blockers and cardioselective calcium channel blockers (eg, verapamil,
diltiazem) may be of benefit, by increasing left ventricular filling time,
improving ventricular relaxation, and decreasing compensatory sympathetic
stimulation. In addition, low-medium dose diuretics lower preload and may
provide symptomatic relief. Small initial doses should be administered to
prevent hypotension because patients are frequently extremely sensitive to
alterations in left ventricular volume. Higher doses may be needed if the
serum albumin level is low secondary to concomitant nephrotic syndrome.
ACEIs and angiotensin II inhibitors are poorly tolerated in patients with
amyloidosis. Even small doses may result in profound hypotension,
probably secondary to an autonomic neuropathy. Beta-blockers and calcium
channel blockers have not been shown to improve day-to-day symptoms or
to favorably alter the natural history in patients with diastolic heart failure.
No published data are available on the use of intravenous (IV) inotropic or
vasodilator drugs.
Patients with a history of atrial fibrillation should be anticoagulated. In
patients with atrial fibrillation, the rate should be controlled adequately.
Removal of the atrial contribution to ventricular filling may worsen the
existing diastolic dysfunction, and a rapid ventricular response may further

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compromise diastolic filling, creating a crisis. Therefore, maintaining sinus

rhythm is important, and medications such as amiodarone and beta-blockers
are often used.
Digoxin should be used with caution because it is potentially
arrhythmogenic, particularly in patients with amyloidosis.
Antiplasma cell therapy with melphalan may slow the progress of systemic
amyloidosis by stopping production of the paraprotein responsible for the
formation of amyloid. The prognosis of patients with primary systemic
amyloidosis remains poor, with a median survival of approximately 2 years
despite intervention with alkylating-based chemotherapy in selected cases.
In specific cases, chemotherapy has dramatic benefits, with improvement in
systemic and cardiac manifestations.
The treatment of Loeffler endocarditis consists of correctly identifying the
condition before the end-stage fibrosis occurs. Medical therapy with
corticosteroids, cytotoxic agents (eg, hydroxyurea), and interferon to
suppress the intense eosinophilic infiltration of the myocardium is
appropriate during the early phase of Loeffler endocarditis and improves
symptoms and survival. Conventional heart failure medication is also given.
Chelation therapy or therapeutic phlebotomy is effective in patients with
hemochromatosis to decrease the iron overload.

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Medical Management

Restrictive cardiomyopathy (RCM) has no specific treatment. However, therapies

directed at individual causes of RCM have been proven to be effective. Examples
of this include corticosteroids for sarcoidosis and Loeffler endocarditis,
endocardiectomy for endomyocardial fibrosis and Loeffler endocarditis,
phlebotomy and chelation for hemochromotosis, and chemotherapy for
amyloidosis. The mainstays of medical treatment include diuretics, vasodilators,

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and angiotensin-converting enzyme inhibitors (ACEs) as indicated, as well as

anticoagulation (if not contraindicated).
In selected patients, permanent pacing, LVAD therapy, and transplantation (heart
or heart-liver) may be considered.

(Wolters Kluwer, 2012)

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NURSING DIAGNOSIS

DECREASED CARDIAC OUTPUT:

Related to negative ionotropic changes in the heart (decreased cardiac contractility)
secondary to cardiac muscle changes.

Desired outcomes:

By atleast 24 hours before hospital discharge, patient exhibits adequate cardiac output as
evidence by SBP at least 90mmHg, HR 100bpm or less, urinary output atleast 30 mL/hr,
stable weight, eupnea, normal breath sounds, and edema +1 or less on a 0-4+ scale. By
atleast 48 hours before hospital discharge, patient is free of new dysrhythmias, does not
exhibit significant changes in mental status and remains oriented to person, place and
time.

Assess for, document, and report evidence of decreased cardiac output, such as
edema, jugular venous distention, adventitious breath sounds, shortness of
breadth, decreased urinary output, extra heart sounds such as S3, changes in

mental status or LOC, cool extremities, hypotension, tachycardia, and tachypnea.

Keep accurate I & O records: weigh patient daily

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Assist with ADL and facilitate coordination of health care providers to ensure rest
periods between care activities to decrease cardiac workload. Allow 90 minutes

for undisturbed rest. If necessary, limit visitors to enable adequate rest.

Administer medications as prescribed, such as beta- blockers, calcium channel

blockers and anti-dysrhythmic agents.

Assist patient into position of comfort, usually semi- Fowlers position, (HOB up
to 30-45 degrees).

(Swearingen, 2007)

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NURSING MANAGEMENT

Administer drugs as ordered to promote adequate heart function.

Assess hemodynamic status every 3 hours or more frequently if necessary
Monitor intake and output more closely and obtain daily weights; institute fluid

restrictions as ordered.
Institute continuous cardiac monitoring to evaluate for arrhythmias.
Assess the patient for possible adverse drug reactions, such as orthostatic
hypotension associated with use of vasodilators, ACE inhibitors, diuretics. Urge

patient to change position slowly.

Be aware that patients with hypertrophic cardiomyopathy should not receive
medication that may decrease preload (diuretics, nitrates) or dopamine or digoxin
because the increase in myocardial contractility may worsen the outflow

obstruction.
Auscultate heart and lung sounds, being alert for S3 and S4 heart sounds or
murmurs, or crackles or rhonchi, and wheezes indicative of heart failure. Monitor

vital signs.
Assist patient with ADLs to decrease oxygen demand.

(Wolters Kluwer, 2012)

Reference:
Merck Manual (2014). Dilated Cardiomyopathy. Retrieved September 14, 2014, from
http://www.merckmanuals.com/professional/cardiovascular_disorders/cardiomyopathies/

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dilated_cardiomyopathy.html

Bott-Silverman, C. (2010). Dilated and Restrictive Cardiomyopathies. Retrieved

September 14, 2014, from
http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/dilat
ed-restrictive-cardiomyopathy/

Kumar, V., Abbas, A., & Aster, J. c. (2013). Robbins Basic Pathology (9th ed.).
Pennsylvania, USA: Elsevier.

http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/dilat
ed-restrictive-cardiomyopathy/

Gene Diagnostics. (2014). Dilated Cardiomyopathies. Retrieved September 14, 2014,

from http://www.genedx.com/wp-content/uploads/crm_docs/90346-clinician-guidedcm-.pdf
Gene Diagnostics. (2014). Hypertrophic Cardiomyopathy. Retrieved September 14,
2014, from http://www.genedx.com/wpcontent/uploads/crm_docs/clinicians_guide_hcm.pdf.pdf

Merck Manuals. (2014). Hypertrophic Cardiomyopathy. Retrieved September 14, 2014,

from
http://www.merckmanuals.com/professional/cardiovascular_disorders/cardiomyopathies/
hypertrophic_cardiomyopathy.html

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Heart Association . (September 31, 2013). Restrictive Cardiomyopathies. Retrieved

September 14, 2014, from https://www.heart.org/idc/groups/heartpublic/@wcm/@hcm/documents/downloadable/ucm_312227.pdf

Swearingen, P. L. (2007). Manual of Medical- Surgical Nursing Care (6th ed.). Missouri,
USA: Mosby Elsevier.

Wolters Kluwer. (2012). Critical Care Nursing Made Incredibly Easy. Pennsylvania,
USA: Lippincott Williams & Wilkins.

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