Cardiomyopathy is a heart muscle disease associated with cardiac dysfunction.

It is classified according
to the structural and functional abnormalities of the heart muscle: dilated cardiomyopathy (DCM),
hypertrophic cardiomyopathy (HCM), restrictive or constrictive cardiomyopathy (RCM), arrhythmogenic
right ventricular cardiomyopathy (ARVC), and unclassified cardiomyopathy (Libby, et al., 2008). A patient
may have pathology representing more than one of these classifications, such as a patient with HCM
developing dilation and symptoms of DCM. Ischemic cardiomyopathy is a term frequently used to
describe an enlarged heart caused by coronary artery disease, which is usually accompanied by heart
failure (see Chapter 30).

Pathophysiology

The pathophysiology of all cardiomyopathies is a series of events that culminate in impaired cardiac
output. Decreased stroke volume stimulates the sympathetic nervous system and the renin–
angiotensin–aldosterone response, resulting in increased systemic vascular resistance and increased
sodium and fluid retention, which places an increased workload on the heart. These alterations can lead
to heart failure (see Chapter 30).

Dilated Cardiomyopathy

DCM is the most common form of cardiomyopathy, with an incidence of 5 to 8 cases per 100,000 people
per year (Libby, et al., 2008). DCM is distinguished by significant dilation of the ventricles without
simultaneous hypertrophy (ie, increased muscle wall thickness) and systolic dysfunction (Fig. 29-8). The
ventricles have elevated systolic and diastolic volumes but a decreased ejection fraction.

More than 75 conditions and diseases may cause DCM, including pregnancy, heavy alcohol intake, viral
infection (eg, influenza), chemotherapeutic medications (eg, daunorubicin [Cerubidine], doxorubicin
[Adriamycin]), and Chagas disease. When the causative factor cannot be identified, the diagnosis is
idiopathic DCM, which accounts for approximately 25% of all heart failure cases. Because genetic factors
may be involved, echocardiography and ECG should be used to screen all first-degree blood relatives (eg,
parents, siblings, children) for DCM (Libby, et al., 2008).

Microscopic examination of the muscle tissue shows diminished contractile elements (actin and myosin
filaments) of the muscle fibers and diffuse necrosis of myocardial cells. The result is poor systolic
function. The structural changes decrease the amount of blood ejected from the ventricle with systole,
increasing the amount of blood remaining in the ventricle after contraction. Less blood is then able to
enter the ventricle during diastole, increasing end-diastolic pressure and eventually increasing
pulmonary and systemic venous pressures. Altered valve function, usually regurgitation, can result from
an enlarged stretched ventricle. Poor blood flow through the ventricle may also cause ventricular or
atrial thrombi, which may embolize to other locations in the body. Early diagnosis and treatment can
prevent or delay significant symptoms and sudden death from DCM.

Hypertrophic Cardiomyopathy
HCM is a rare autosomal dominant condition, occurring in men, women, and children (often detected
after puberty) with an estimated prevalence rate of 0.05% to 0.2% of the population in the United States
(Zevitz, 2006). Echocardiograms may be performed every year from 12 to 18 years of age and then every
5 years from 18 to 70 years of age in susceptible individuals. Doppler echocardiography may also be
used to detect the HCM and blood flow alterations (Libby, et al., 2008). HCM also may be idiopathic (ie,
no known cause).

In HCM, the heart muscle asymmetrically increases in size and mass, especially along the septum (see
Fig. 29-8). HCM often affects nonadjacent areas of the ventricle. The increased thickness of the heart
muscle reduces the size of the ventricular cavities and causes the ventricles to take a longer time to
relax after systole. During the first part of diastole it is more difficult for the ventricles to fill with blood.
The atrial contraction at the end of diastole becomes critical for ventricular filling and systolic
contraction.

Cardiac muscle cells normally lie parallel to and end-toend with each other. The hypertrophied cardiac
muscle cells are disorganized, oblique, and perpendicular to each other, decreasing the effectiveness of
contractions and possibly increasing the risk of dysrhythmias such as ventricular tachycardia and
ventricular fibrillation. In HCM the coronary arteriole walls are thickened, which decreases the internal
diameter of the arterioles. The narrow arterioles restrict the blood supply to the myocardium, causing
numerous small areas of ischemia and necrosis. The necrotic areas of the myocardium ultimately fibrose
and scar, further impeding ventricular contraction.

Restrictive Cardiomyopathy

RCM is characterized by diastolic dysfunction caused by rigid ventricular walls that impair diastolic filling
and ven tricular stretch (see Fig. 29-8). Systolic function is usually normal. RCM may be associated with
amyloidosis (amyloid, a protein substance, is deposited within cells) and other such infiltrative diseases.
However, the cause is unknown (ie, idiopathic) in most cases. Signs and symptoms are similar to
constrictive pericarditis: dyspnea, nonproductive cough, and chest pain. Echocardiography, as well as
measurement of pulmonary artery systolic (PAS) pressure, pulmonary artery wedge pressure (PAWP),
and central venous pressure (CVP) are used to differentiate the two conditions.

Arrhythmogenic Right Ventricular Cardiomyopathy

ARVC occurs when the myocardium of the right ventricle is progressively infiltrated and replaced by
fibrous scar and adipose tissue. Initially, only localized areas of the right ventricle are affected, but as the
disease progresses, the entire heart is affected. Eventually, the right ventricle dilates and develops poor
contractility, right ventricular wall abnormalities, and dysrhythmias. The prevalence of ARVC is unknown
because many cases are not recognized. Palpitations or syncope may develop between 15 and 40 years
of age. ARVC should be considered in patients with ventricular tachycardia originating in the right
ventricle (ie, a left bundle branch block configuration on ECG) or sudden death, especially among young
athletes (Libby, et al., 2008). ARVC is genetic (ie, autosomal dominant) (Sen-Chowdhry, Syrris &
Mckenna, 2005). First-degree blood relatives (eg, parents, siblings, children) should be screened for the
disease with a 12-lead ECG, Holter monitor, and echocardiography.
Unclassified Cardiomyopathies

Unclassified cardiomyopathies are different from or have characteristics of more than one of the
previously described types. Examples of unclassified cardiomyopathies include fibroelastosis,
noncompacted myocardium, systolic dysfunction with minimal dilation, and mitochondrial involvement
(Libby, et al., 2008)

Clinical Manifestations

Patients with cardiomyopathy may remain stable and without symptoms for many years. As the disease
progresses, so do the symptoms. Frequently, dilated or restrictive cardiomyopathy is first diagnosed
when the patient presents with signs and symptoms of heart failure (eg, dyspnea on exertion, fatigue).
Patients with cardiomyopathy may also report PND, cough (especially with exertion), and orthopnea,
which may lead to a misdiagnosis of bronchitis or pneumonia. Other symptoms include fluid retention,
peripheral edema, and nausea, which is caused by poor perfusion of the gastrointestinal system. The
patient also 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 (Libby, et al., 2008; Zevitz, 2006).

Regardless of type and cause, cardiomyopathy may lead to severe heart failure, lethal dysrhythmias, and
death. The mortality rate is highest for African Americans and the elderly.

Assessment and Diagnostic Findings

Physical examination at early stages may reveal tachycardia and extra heart sounds (eg, S3, S4). Patients
with DCM may have diastolic murmurs, and patients with DCM and HCM may have systolic murmurs.
With disease progression, examination also reveals signs and symptoms of heart failure (eg, crackles on
pulmonary auscultation, jugular vein distention, pitting edema of dependent body parts, enlarged liver).

Diagnosis is usually made from findings disclosed by the patient history and by ruling out other causes of
heart failure such as myocardial infarction. The echocardiogram is one of the most helpful diagnostic
tools because the structure and function of the ventricles can be observed easily. Cardiac MRI may also
be used, particularly to assist with the diagnosis of HCM (Rickers, Wilke, Jerosch-Herold, et al., 2005).
ECG demonstrates dysrhythmias (atrial fibrillation, ventricular dysrhythmias) and changes consistent
with left ventricular hypertrophy (left axis deviation, wide QRS, ST changes, inverted T waves). In ARVC,
there often is a small deflection, an epsilon wave, at the end of the QRS. The chest x-ray reveals heart
enlargement and possibly pulmonary congestion. Cardiac catheterization is sometimes used to rule out
coronary artery disease as a causative factor. Endomyocardial biopsy may be performed to analyze
myocardial cells.

Medical Management

Medical management is directed toward identifying and managing possible underlying or precipitating
causes; correcting the heart failure with medications, a low-sodium diet, and an exercise/rest regimen
(see Chapter 30); and controlling dysrhythmias with antiarrhythmic medications and possibly with an
implanted electronic device, such as an implantable cardioverter defibrillator (see Chapter 27). Systemic
anticoagulation to prevent thromboembolic events is usually recommended. If the patient has signs and
symptoms of congestion, fluid intake may be limited to 2 L each day. Patients with HCM should avoid
dehydration and may need beta-blockers (atenolol [Tenormin], metoprolol [Lopressor], nadolol
[Corgard], propranolol [Inderal]) to maintain cardiac output and minimize the risk of left ventricular
outflow tract obstruction during systole. Patients with HCM or RCM may need to limit physical activity to
avoid a life-threatening dysrhythmia.

A pacemaker may be implanted to alter the electrical stimulation of the muscle and prevent the forceful
hyperdynamic contractions that occur with HCM. Atrial-ventricular and biventricular pacing have been
used to decrease symptoms and obstruction of the left ventricular outflow tract. For some patients with
DCM and HCM, biventricular pacing increases the ejection fraction and reverses some of the structural
changes in the myocardium.

Nonsurgical septal reduction therapy, also called alcohol septal ablation, has been used to treat
obstructive HCM. In the cardiac catheterization laboratory, a percutaneous catheter is positioned in one
or more of the septal coronary arteries. Once the position is verified, 1 to 5 mL of 96% to 98% ethanol
(ethyl alcohol) is injected at a rate of about 1 mL/min to destroy the myocardial cells; it is believed that
the ethanol causes dehydration of the cardiac cells (Libby, et al., 2008; Zevitz, 2006). The slow rate of
injection minimizes the risk of heart block and premature ventricular contractions. The procedure
produces a septal myocardial infarction. The resulting scar is thinner than the living myocardium had
been, so the obstruction is decreased. The patient may develop a left anterior hemibranch block or left
bundle branch block. If the patient experiences pain, hydrocodone/ acetaminophen (Vicodin) is usually
administered. Nitrates and morphine are not used because coronary artery dilation is contraindicated.

Surgical Management

When heart failure progresses and medical treatment is no longer effective, surgical intervention,
including heart transplantation, is considered. However, because of the limited number of organ donors,
many patients die waiting for transplantation. In some cases, a left ventricular assist device is implanted
to support the failing heart until a suitable donor heart becomes available.

Left Ventricular Outflow Tract Surgery

When patients with HCM become symptomatic despite medical therapy and a difference in pressure of
50 mm Hg or more exists between the left ventricle and the aorta, surgery is considered. The most
common procedure is a myectomy (sometimes referred to as a myotomy-myectomy), in which some of
the heart tissue is excised. Septal tissue approximately 1-cm wide and deep is cut from the enlarged
septum below the aortic valve. The length of septum removed depends on the degree of obstruction
caused by the hypertrophied muscle.

Instead of a septal myectomy, the surgeon may open the left ventricular outflow tract to the aortic valve
by mitral valvuloplasty involving the leaflets, chordae, or papillary muscles, or the patient’s mitral valve
may be replaced with a low-profile disk valve. The space taken up by the mitral valve is substantially
reduced by the valvuloplasty or prosthetic valve, allowing blood to move around the enlarged septum to
the aortic valve through the area the mitral valve once occupied. The primary complication of all the
procedures is dysrhythmia. Additional complications include postoperative surgical complications such
as pain, ineffective airway clearance, deep vein thrombosis, risk of infection, and delayed surgical
recovery.

Latissimus Dorsi Muscle Wrap

DCM may be treated with a latissimus dorsi muscle wrap, also called dynamic cardiomyoplasty (Libby, et
al., 2008; Woods, Froelicher, Motzer, et al., 2005). The left latissimus dorsi muscle is dissected from the
lateral side and back of the chest, leaving the medial end of the muscle and the blood supply intact, and
the lateral end of the muscle is pulled through the pleural space into the pericardium. The latissimus
dorsi muscle flap is then wrapped around the ventricles and sutured in place. Pacemaker leads are
implanted into the muscle flap, and a pacemaker generator is implanted in the chest wall. For at least 2
weeks following surgery, the pacemaker remains turned off to facilitate the development of adhesions
between the muscle wrap and the ventricles. Eventually, the pacemaker is turned on and used to
stimulate latissimus dorsi muscle contraction. Several weeks of training, or conditioning, are necessary
before this skeletal muscle functions effectively. The ultimate goal is for the latissimus dorsi muscle wrap
to augment ventricular contraction and increase cardiac output. This skeletal muscle loses its
contractility over time, and the patient may be evaluated for heart transplantation.

Heart Transplantation

Because of advances in surgical techniques and immunosuppressive therapies, heart transplantation is

now a therapeutic option for patients with end-stage heart disease. Cyclosporine (Gengraf, Neoral,
Sandimmune) is an immunosuppressant that greatly decreases the body’s rejection of foreign proteins,
such as transplanted organs. Unfortunately, cyclosporine also decreases the body’s ability to resist
infections, and a satisfactory balance must be achieved between suppressing rejection and avoiding
infection.

Cardiomyopathy, ischemic heart disease, valvular disease, rejection of previously transplanted hearts,
and congenital heart disease are the most common indications for transplantation (Fuster, et al., 2008;
Libby, et al., 2008; Moser & Riegel, 2008). Typical candidates have severe symptoms uncontrolled by
medical therapy, no other surgical options, and a prognosis of less than 2 years to live. A
multidisciplinary team screens the candidate before recommending the transplantation procedure
(Chart 29-1). The person’s age, pulmonary status, other chronic health conditions, psychosocial status,
family support, infections, history of other transplantations, compliance, and current health status are
considered.

When a donor heart becomes available, a computer generates a list of potential recipients on the basis
of ABO blood group compatibility, the body sizes of the donor and the potential recipient, age, severity
of illness, length of time on the waiting list, and the geographic locations of the donor and potential
recipient. Distance is a factor because postoperative function depends on the heart being implanted
within 4 hours of harvest from the donor. Some patients are candidates for more than one organ
transplant (eg, heart–lung, heart–kidney, heart–liver).

Orthotopic transplantation is the most common surgical procedure for cardiac transplantation (Fig. 29-
9). The recipient’s heart is removed, and the donor heart is implanted at the vena cava and pulmonary
veins. Some surgeons prefer to remove the recipient’s heart, leaving a portion of the recipient’s atria
(with the vena cava and pulmonary veins) in place. The donor heart, which usually has been preserved in
ice, is prepared for implant by cutting away a small section of the atria that corresponds with the
sections of the recipient’s heart that were left in place. The donor heart is implanted by suturing the
donor atria to the residual atrial tissue of the recipient’s heart. After the venous or atrial anastomoses
are complete, the recipient’s pulmonary artery and aorta are sutured to those of the donor heart.

Patients who have had heart transplants are constantly balancing the risk of rejection with the risk of
infection. They must adhere to a complex regimen of diet, medications, activity, follow-up laboratory
studies, biopsies of the transplanted heart (to diagnose rejection), and clinic visits. Most commonly,
patients receive tacrolimus (Prograf) or cyclosporine, mycophenolate mofetil (CellCept) or azathioprine
(Imuran), and corticosteroids (eg, prednisone) to minimize rejection.

The transplanted heart has no nerve connections (ie, denervated heart) to the recipient’s body, so the
sympathetic and vagus nerves do not affect the transplanted heart. The resting rate of the transplanted
heart is approximately 70 to 90 bpm (beats per minute), but it increases gradually if catecholamines are
in the circulation. Patients must gradually increase and decrease their exercise (ie, extended warm-up
and cool-down periods), because 20 to 30 minutes may be required to achieve the desired heart rate.
Atropine does not increase the heart rate of transplanted hearts.

In addition to rejection and infection, complications may include accelerated atherosclerosis of the
coronary arteries (ie, cardiac allograft vasculopathy, accelerated graft atherosclerosis, transplant
coronary artery disease). Both immunologic and nonimmunologic factors cause arterial injury and
inflammation of the coronary arteries. The arterial smooth muscle proliferates and there is hyperplasia
of the coronary artery intima, accelerating atherosclerosis along the entire length of the coronary
arteries (Libby, et al., 2008; Woods, et al., 2005). Hypertension may occur in patients taking cyclosporine
or tacrolimus; the cause has not been identified. Osteoporosis is a frequent side effect of the
antirejection medications as well as pretransplantation dietary insufficiency and medications. Patients
with a long-term sedentary lifestyle are at greater risk for osteoporosis. Posttransplantation
lymphoproliferative disease and cancer of the skin and lips are the most common malignancies after
transplantation, possibly caused by immunosuppression. Weight gain; obesity; diabetes; dyslipidemias
(eg, hypercholesterolemia); hypotension; renal failure; and central nervous system, respiratory, and
gastrointestinal disturbances may be adverse effects of the corticosteroids or other
immunosuppressants. Toxicity from immunosuppressant medications may occur. The 1-year survival
rate for patients with transplanted hearts is approximately 81% to 95%, and the 10-year survival rate is
approximately 50% to 70% (Fuster, et al., 2008; Libby, et al., 2008; Moser & Reigel, 2008).
 In the first year after transplant, patients respond to the psychosocial stresses imposed by organ
transplantation in various ways. Most report a better quality of life after transplantation. Some
experience guilt that someone had to die for them to be able to live, have anxiety about the new heart,
experience depression or fear about rejection, or have difficulty with family role changes before and
after transplantation (Fuster, et al., 2008; Kaba, Thompson, Burnard, et al., 2005; Moser & Reigel, 2008).
After the first year, many patients believe that their lives have purpose, they are connected to a force
greater than themselves, and look forward to the future (Flattery, Salyer, Maltby, et al., 2006).

Mechanical Assist Devices and Total Artificial Hearts

The use of cardiopulmonary bypass in cardiovascular surgery and the possibility of performing heart
transplantation in patients with end-stage cardiac disease, as well as the desire for a treatment option
for patients with such disease who are not transplant candidates, have increased the need for
mechanical assist devices. Patients who cannot be weaned from cardiopulmonary bypass and patients in
cardiogenic shock may benefit from a period of mechanical heart assistance. The most commonly used
device is the intra-aortic balloon pump (see Chapter 30). This pump decreases the work of the heart
during contraction but does not perform the actual work of the heart

Ventricular Assist Devices

More complex devices that actually perform some or all of the pumping function for the heart also are
being used. These more sophisticated ventricular assist devices (VADs) can circulate as much blood per
minute as the heart, if not more (Fig. 29-10). Each VAD is used to support one ventricle. Some VADs can
be combined with an oxygenator; the combination is called extracorporeal membrane oxygenation
(ECMO). The oxygenator–VAD combination is used for the patient whose heart cannot pump adequate
blood through the lungs or the body.

VADs may be used as (1) a “bridge to recovery” for patients who require temporary assistance for
reversible ventricular failure, (2) a “bridge to transplant” for patients with end-stage heart failure until a
donor organ becomes available for transplant (most common), and (3) “destination therapy” for
patients with end-stage heart failure who are not candidates for or decline heart transplantation. When
VADs are used as destination therapy, patients are discharged from the hospital with the devices in
place (Fuster, et al., 2008; Libby, et al., 2008; Moser & Reigel, 2008).

ADs may be external, internal (ie, implanted) with an external power source, or completely internal, and
they may generate a pulsatile or continuous blood flow. There are three types of VADs: pneumatic,
electric or electromagnetic, and axial flow. Pneumatic VADs are external or implanted pulsatile devices
with a flexible reservoir housed in a rigid exterior. The reservoir usually fills with blood drained from the
atrium or ventricle. The device then forces pressurized air into the rigid housing, compressing the
reservoir and returning the blood to the circulation, usually into the aorta. Electric or electromagnetic
VADs are similar to the pneumatic VADs, but instead of using pressurized air to return the blood to the
circulation, one or more flat metal plates are pushed against the reservoir. Axial flow VADs use a rotary
mechanism (an impeller) to create a nonpulsatile blood flow. The impeller spins rapidly within the VAD,
creating a vacuum that pulls blood into the VAD and then pushes the blood out into the systemic
circulation—the process is similar to a fan spinning in a tunnel, pulling air in one end of the tunnel and
pushing it out the other.

Total Artificial Hearts

Total artificial hearts are designed to replace both ventricles. Some require the removal of the patient’s
heart to implant the total artificial heart and others do not. One total artificial heart has been approved
for use in the United States. Although there has been some short-term success, the long-term results
have been disappointing. Researchers hope to develop a device that can be permanently implanted and
that will eliminate the need for donated human heart transplantation for end-stage cardiac disease
(Libby, et al., 2008; Moser & Reigel, 2008).

Complications of VADs and total artificial hearts include bleeding disorders, hemorrhage, thrombus,
emboli, hemolysis, infection, renal failure, right-sided heart failure, multisystem failure, and mechanical
failure (Libby, et al., 2008; Moser & Reigel, 2008). Nursing care of patients with these mechanical assist
devices focuses on assessment for and minimization of these complications as well as providing
emotional support and education about the device as well as the underlying cardiac disease.