cardio mio path y

46 nursing standard february 18/vol18/no23/2004

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Cardiomyopathy

The aim of this article is to provide an overviewof the aetiology, treatment and clinical presenta-tion of patients with cardiomyopathy and describethe role of the nurse in patient management. Italso aims to highlight the psychosocial aspectsthat are a part of the disease and the ethical issuesthat arise in relation to familial evaluation andgenetic testing. After reading this article you shouldbe able to:� Recognise the difference between the types of

cardiomyopathy.� Be aware of the prevalence and aetiology of the

disease.� Realise the genetic inheritance of the condition

and the importance of family screening.� Understand the mechanism and risk stratifica-

tion for sudden death. � Identify current treatment trends for the various

cardiomyopathies.� Describe the nurse’s role in the management of

cardiomyopathy.

Cardiomyopathies are defined as diseases of themyocardium associated with cardiac dysfunction,and are classified as (Richardson et al 1996):� Dilated cardiomyopathy (DCM).� Hypertrophic cardiomyopathy (HCM).� Arrhythmogenic right ventricular cardiomyopathy

(ARVC).� Restrictive cardiomyopathy (RCM).This article describes the various types of car-diomyopathies, their physiology and treatment, anddiscusses the psychological impact that the diag-nosis of cardiomyopathy can bring – informationon patient support is also provided. Although thereare many similarities with each type of cardiomy-opathy, there are also distinct differences.

Dilated cardiomyopathy DCM is characterisedby dilation and impaired contraction of the left ven-tricle (Figure 1) or both ventricles. Although in manycases the aetiology is not known, many factorsappear to be associated with its development, forexample, myocarditis, alcohol, metabolic disorders,infiltrative disorders, autoimmune disease, preg-nancy and familial/genetic factors (Richardson et al1996). It may also be associated with recognisedcardiovascular disease in which the degree of myocar-dial dysfunction is not explained by the presentingpathology or the extent of ischaemic damage(Richardson et al 1996). Hypertrophic cardiomyopathy HCM is charac-terised by left and/or right ventricular hypertrophy,which is usually asymmetric and involves the inter-ventricular septum (Figure 2). Typically, the left ven-tricular volume is normal or reduced, and is oftenassociated with left ventricle outflow tract gradi-ent. Arrhythmias and premature sudden death arecommon (Maron et al 1995). HCM is the most com-mon cause of sudden death in people under theage of 30, and accounts for a high proportion ofathlete-associated sudden deaths (Maron et al 1995).It occurs equally in both sexes and all races (Sorajjaet al 2000). Although HCM is a chronic diseasewithout a known cure, a number of treatments areavailable to alter its course.What causes HCM? HCM is a familial disease withan autosomal dominant inheritance pattern, whichmeans that the condition may be passed from onegeneration to the next and does not skip a gener-ation (McKenna and Behr 2002). The unexplained

Different types of cardiomyopathy

Introduction

Aim and intended learning outcomes

NS229 Cruickshank S (2004) Cardiomyopathy. Nursing Standard. 18, 23, 46-52.Date of acceptance: February 21 2003.

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Cardiomyopathypages 46-52

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AuthorStephanie Cruickshank RN, is Cardiomyopathy NurseSpecialist, The Heart Hospital,London. Email: [email protected]

SummaryCardiomyopathy is a disease ofthe heart muscle. There are four main types of cardiomyopathy and each canaffect people differently. Thisarticle discusses the signs andsymptoms, diagnosis and treat-ment for the different types ofthe disease, the importance ofgenetic screening and riskstratification, and the nurse’srole in patient management.

Key words� Cardiovascular system and

disorders� Heart disorders� Heart disorders: nursing

These key words are basedon subject headings from theBritish Nursing Index. Thisarticle has been subject todouble-blind review.

TIME OUT 1Make notes on the main types ofcardiomyopathy and how they differ.Discuss your answers with a colleague.

In brief

Heart disorders

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february 18/vol18/no23/2004 nursing standard 47

feature of the disease is that while some casesappear to affect the whole of the left ventricle,others may be confined to a small area of the ven-tricle. The disease expression in a family may varyfrom person to person, with some people severelyaffected and others less so. With the recent advancesin genetics, gene carriers have been identified whodo not have HCM but have the potential to pass iton to future generations.Arrhythmogenic right ventricular cardiomy-opathy ARVC is a familial myocardial disease char-acterised pathologically by right ventricular (RV)myocardial atrophy and fibrofatty replacement,which means the heart muscle is gradually replacedby fatty tissue. Areas of fatty tissue may lead toweakness and bulges in the cardiac muscle wall(Figure 3). It is a progressive heart muscle diseasethat, with time, may lead to more diffuse RV involve-ment and left ventricular (LV) changes, and mayculminate in heart failure (Hamid 2001).

ARVC predominantly presents in adolescents oryoung adults, but it is increasingly being diagnosedin an older age group – estimated figures of theincidence of ARVC range between 1:3,000 and1:10,000 (Hamid 2001). It is a diagnosis that shouldbe considered when presented with a patient whohas ventricular tachycardia of left bundle branchmorphology – assessment is by means of a routineECG. It may cause sudden cardiac death in a youngperson with what is considered to be a structurallynormal heart (Hamid 2001).What causes ARVC? A familial background has beendemonstrated in nearly 50 per cent of ARVC cases,with an autosomal dominant pattern of inheritance(Hamid 2001). As yet, the involved genes and themolecular defects remain unknown, but researchcontinues in this area. In some patients there iseither no evidence of inheritance or there is insuf-ficient information about the individual’s family toassess inheritance.

A further difficulty is that in a family, features ofthe disease may be variable, and the disease mayappear to skip a generation.Restrictive cardiomyopathy The fourth and leastcommon type of cardiomyopathy is restrictive cardio-myopathy (RCM). RCM is defined as a heart mus-cle disease that results in impaired ventricular filling,with normal or decreased diastolic volume of eitheror both ventricles (Richardson et al 1996). Systolicfunction usually remains normal, at least early inthe disease, and wall thickness may be normal orincreased, depending on the underlying cause(Richardson et al 1996). The condition usually resultsfrom increased stiffness of the myocardium thatcauses pressure in the ventricle (or ventricles) to riseprecipitously with only small increases in volume.As the condition affects either or both ventricles,the patient may present with signs of left or rightventricular failure.

RCM may be a primary disorder due to endomy-ocardial fibrosis, Loeffler’s cardiomyopathy or

idiopathic cardiomyopathy (aetiology unknown)(Richardson et al 1996). Secondary RCM can bedue to infiltrative disease such as amyloidosis, post-irradiation therapy or storage diseases such ashaemochromatosis, glycogen storage disease orFabry’s disease (Ammash et al 2000).

Idiopathic RCM is sometimes familial and seemsto be associated with skeletal myopathies, whichmay affect only the distal limbs (Fitzpatrick et al1990). In childhood RCM may be more common ingirls and has a poor prognosis compared with RCMin adults (Ammash et al 2000).

Heart disorders

Figure 1. Dilated heart

Figure 2. Hypertrophic cardiomyopathy

Asymmetric septal hypertrophy without obstruction

LALARARA

LVRVLVRV

Normal heart Dilated cardiomyopathy

Mitral valve

Aortic valve

Reduced leftventricle

Enlarged intraventricularseptum

Pulmonaryvalve

Tricuspid valve

RA = right atrium LA = left atriumRV = right ventricle LV = left ventricle

RA

RV

LA

LV

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The clinical signs and symptoms of all types of car-diomyopathy (Table 1) are established most easilyand reliably with two dimensional echocardiogra-phy and ECG. In the presence of any abnormalityfurther investigations may be required to securethe diagnosis and to plan treatment management.DCM Patients commonly present with signs andsymptoms of heart failure, pulmonary congestionand/or low cardiac output, often with a backgroundof exertional symptoms and fatigue for many monthsor years before their diagnosis (McKenna and Behr2002). Symptoms may include dyspnoea, chest pain,embolic events such as atrial fibrillation, syncopeand palpitations. An acute illness or the develop-ment of arrhythmia, in particular atrial fibrillation,may precipitate acute symptoms and prompt theindividual to seek medical attention. Sometimes apatient is diagnosed as a result of family or routinemedical screening, which is why it is important totake a careful family history, as family screening

accounts for a large number of patient referrals.Uncommonly, the patient may present with systemicembolism or sudden death (Elliott 2000).HCM Patients can present at any age with dysp-noea, chest pain, unexplained syncope, arrhythmiaor sudden death (McKenna and Behr 2002). Manypatients have no or only minor symptoms and inchildren and adolescents the diagnosis is often madeduring family screening. Exertional chest pain occursin up to 30 per cent of adults, and many complainof atypical pain that is prolonged and occurs at restand after meals (Gilligan et al 1991). Dyspnoea iscommon in adult patients, probably as a conse-quence of elevated pulmonary venous pressureresulting from impaired ventricular relaxation andfilling (McKenna and Behr 2002). Patients less com-monly present with symptoms such as waking upat night breathless and orthopnoeic, and requiretwo or more pillows to sleep at night as a result.Approximately 15-25 per cent of patients experi-ence syncope and 20 per cent complain of pre-syncope (Maron 1997). Palpitations are a frequentcomplaint (Maron 1997).ARVC The patient may be asymptomatic or maypresent with palpitations, syncope, presyncope,lethargy, dyspnoea and oedema (Hamid 2001). Aswith HCM the severity of symptoms and risk ofcomplications vary greatly between people andmany never experience any serious problems relatedto their condition. RCM The underlying cause of RCM may not be obvi-ous on presentation – the patient may present withsymptoms similar to those of DCM. Exercise intol-erance is a frequent symptom (Sudhir 1997).Uncommonly, the first presentation may be suddencardiac death. Other symptoms include dyspnoea,paroxysmal nocturnal dyspnoea, orthopnoea, periph-eral oedema, ascites, fatigue and weakness (Sudhir1997). Angina does not occur except in amyloido-sis, in which it may be the presenting symptom(Hesse 1993). In advanced cases, the patient maypresent with all the signs of heart failure exceptcardiomegaly. Up to one third of patients with idio-pathic RCM may present with thromboembolic com-plications such as atrial fibrillation (Hirota 1990).

Investigations for cardiomyopathy are similar whichevertype is suspected; however, as Table 2 indicates,some tests are more specific for a certain form.DCM A routine ECG, echocardiogram and chest X-ray

Diagnosis

Signs and symptoms

Heart disorders

TIME OUT 2Sarah, a 26-year-old woman, presents toA&E following a cardiac arrest. Aftersuccessful resuscitation she is diagnosedwith hypertrophic cardiomyopathy. Whatinvestigations would be carried out tosecure the diagnosis?

Figure 3. Arrhythmogenic right ventricular cardiomyopathy

LA

LV

RA

RV

Fatty tissue may lead to weaknessand bulges in the heart muscle wall

Dilation of right ventricle

LA

LV

RA

RV

Fatty tissue

Bulges inventricular wall

Table 1. Signs and symptoms

Type of cardiomyopathy

Dilated Hypertrophic Arrhythmogenic Restrictive

Dyspnoea Yes Yes Yes Yes

Syncope Yes Yes Yes Yes

Sudden death Yes Yes Yes Uncommon

Palpitations Yes Yes Yes Yes

Chest pain Yes Yes Yes Yes

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are usually performed to confirm the diagnosis. Thepatient may go on to have further tests, such as anexercise test, cardiac catheterisation and blood tests.HCM A detailed and accurate family history is impor-tant, especially noting any sudden unexplaineddeaths. The patient may be diagnosed as a resultof family screening or as an incidental finding dur-ing a medical examination. Adults are often asymp-tomatic so in the majority of patients the physicalexamination may be unremarkable; however, inthese asymptomatic patients, estimated mortalityfrom HCM is still 1-2 per cent a year (Elliott et al2000). Echocardiography may show a thickenedleft ventricle. The normal thickness is 12mm or less– in HCM it may be 15mm or more (Elliott 2000 etal). It is important to establish the diagnosis earlyand assess the risk of sudden cardiac death so thatthe appropriate treatment can be given. Once adiagnosis of HCM has been made, it is vitally impor-tant that first-degree relatives of an affected per-son are offered cardiac evaluation.ARVC This might be difficult to diagnose as thepatient may be asymptomatic until the first pres-entation with cardiac arrest. Also the diagnosis maybe missed and the patient may present in later yearswith congestive heart failure with or without ven-tricular arrhythmia.

The inheritance pattern on HCM is the dominanttrait, which means there is a 50 per cent chance oftransmission to offspring.HCM Most patients with HCM have at least one otheraffected relative such as a parent or sibling (McKennaand Behr 2002). Therefore, it is important that all first-degree relatives have a 12-lead ECG and an echocar-diogram performed, as HCM may be present withoutcausing symptoms. The expression of disease is age-related, occurring during or soon after periods ofrapid growth. Cardiovascular abnormalities are com-monly detected during adolescence and children ofan affected individual should undergo cardiac eval-uation at six-monthly intervals until they reach adult-hood; thereafter if the disease is ‘late-onset’ evaluationshould be performed every five years. Genetic test-ing is only available at research centres, but excitingprogress has been made and it is hoped that, in thefuture, genetic evaluation will be available to all.

All patients should undergo non-invasive risk factorstratification with a clinical history, holter monitoring(24-hour continuous, portable ECG recording), andmaximal exercise testing, regardless of symptomaticstatus or the apparent severity of morphologicaldisease (McKeena and Behr 2002).HCM Sudden and unexpected death is the mostdevastating and unpredictable complication of HCM,but only a minority of patients are actually at risk,therefore it is important that all patients undergonon-invasive risk factor stratification with a clinicalhistory, holter monitoring, and maximal exercisetesting regardless of symptomatic status (McKennaand Behr 2002). Box 1 provides the risk factors thatmay lead to sudden death.

Risk stratification, which identifies who is at highrisk of sudden death, allows reassurance to be givento low-risk individuals, while high-risk patients canreceive prophylactic intervention. Patients identi-fied to be at high risk from sudden cardiac deathshould be offered prophylactic implantation of animplantable cardioverter defibrillator (ICD) and/ortreatment with amiodarone (Box 2). An ICD is adevice that uses similar technology to a pacemakerand is inserted under the skin in the chest area. Ifthe patient develops a life-threatening arrhythmia,the device will recognise this and deliver a smallelectrical shock internally to restore normal rhythm.Patients who are at high risk should also be advisedto avoid strenuous exercise or competitive sports,which require extreme physical exertion (McKennaand Behr 2002). Patients with restrictive car-diomyopathy may have undergone similar investi-gations; however with restrictive physiology thepatient is more likely to progress to heart failurerather than sudden death.

Risk stratification for sudden death

Genetic inheritance and family screening

Heart disorders

TIME OUT 3How is cardiomyopathy inherited? Why isit important to take a detailed familyhistory from the patient?

Table 2. Investigations



Previous history � � � �

Echocardiogram � � � �

ECG � � � �

Chest X-ray (to � Possiblyexclude other causesof breathlessness) Physical examination � � � �

Exercise test � � � �

Viral studies �

Endobiopsy � �

24-hour holter � � � �

Magnetic resonance �

imaging/computed tomography (MRI/CT)Cardiac catheterisation � PossiblyScreening � � � �

Signal average ECG � �

TIME OUT 4What risk factors are associated withsudden death in HCM? Whichinvestigations should be carried out toassess this risk?

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ARVC Risk stratification is an important part ofpatient management with the intention of pre-venting arrhythmic sudden death. Important clinicalmarkers include a history of syncope, a malignantfamily history, diffuse right ventricular dilation witha reduction in ejection fraction and patients withleft ventricular involvement (McKenna and Behr2002). Sudden death may be the first manifestationof the disease, mostly in previously asymptomaticyoung adults and athletes.

A fundamental goal of treatment in cardiomyopa-thy is the alleviation of symptoms and the preven-tion of sudden cardiac death (Table 3).DCM This disease has many causes and it is impor-tant that it is detected in the early stages so thatthe patient can receive the most appropriate ther-apy. Recent advances in understanding the patho-physiology of the disease and treatments havesubstantially improved the outlook for many patients.

The primary aim of treatment is to control symp-toms and to prevent disease progression and com-plications, such as progressive heart failure,thromboembolism and sudden death (Elliott 2000).Diuretics and angiotensin-converting enzyme (ACE)inhibitors are used to treat congestive symptoms

and are prescribed for patients with DCM irrespec-tive of the severity of heart failure. These drugsimprove dyspnoea and exercise tolerance, and reducehospitalisation rates and cardiovascular mortality(Elliott 2000). They have also been shown to pre-vent or slow down disease progression in asymp-tomatic patients (Elliott 2000). Angiotensin II receptorantagonists may be prescribed in the case of intol-erable side effects from ACE therapy, as they havea similar therapeutic effect (Elliott 2000). One of themajor concerns for patients with DCM is the risk ofsudden death. Recent work has demonstrated sub-stantial reductions in sudden death and death fromprogressive heart failure in patients treated withbeta-blockers (Elliott 2000) – the initial dose shouldbe low and careful monitoring and titration is required.Another drug used is spironolactone, which hasbeen shown to reduce the risk of sudden death by30 per cent in patients with symptoms of moder-ate to severe heart failure (Elliott 2000). Anticoagulationmay be required in patients with dilated atrial cham-bers and those with atrial fibrillation. Care shouldbe taken when prescribing anti-arrhythmic drugs inDCM, as some may be pro-arrhythmic, that is, theymay precipitate arrhythmias.Multisite ventricular pacing Dual chamber pacing hasbeen advocated as a method for restoring atrium/ventricle (AV) synchrony and improving left ventric-ular co-ordination in patients with severe conges-tive heart failure. Biventricular pacing has been usedwith success, especially in patients with a QRS greaterthan 150ms and a prolonged PR interval, and symp-toms refractory to conventional medical treatment(Elliott 2000).

Patients who are at high risk of sudden death mayrequire an ICD to restore normal heart rhythm (Box2). DCM accounts for up to 50 per cent of refer-

Treatment options

Heart disorders

TIME OUT 5Sarah develops hyperthyroidism as a result of amiodarone therapy. Theoption of an ICD is discussed. What arethe common fears of patients receivingan ICD? How might you help her toovercome these?

Implantable cardioverter defibrillators (ICDs)have been proven to prolong survival in high-risk cardiac patients. An ICD is arelatively small device that is implanted underthe skin, most commonly in the upper chest.It consists of a battery (lasting three-six years),energy delivery components and electroniccircuitry, which are all sealed in one case. TheICD is connected to the heart via one or moreelectrodes that are inserted into veins.

Through these electrodes, the ICD monitorsthe patient’s heart rate and rhythm and candeliver corrective electrical treatment asappropriate. The ICD can deliver high-energytherapy (shocks) to correct serious life-threatening rapid and sustainedarrhythmias. The patient may feel this therapyas a ‘kick in the chest’ or he or she may loseconsciousness before the shock is delivered. In some patients the ICD is used inconjunction with anti-arrhythmic drugs.

Box 2. Implantable cardioverter defibrillator

Box 1. Recognised markersof increased risk of suddendeath in HCM

� Previous cardiac arrest

� Non-sustained ventriculartachycardia on holter orexercise

� Abnormal exertional bloodpressure response

� Unexplained syncope

� Family history of prematuresudden death

� Severe left ventricularhypertrophy >30mm

(McKenna and Behr 2002)

Table 3. Treatments used in cardiomyopathy



Angiotensin- Yes Considered Yesconverting enzyme (ACE)inhibitorsAll antagonists Yes Considered YesDiuretics Yes Considered YesSurgery YesBeta-blockers Yes Yes Yes YesImplantable Considered Considered Consideredcardioverterdefibrillator(ICD) insertionTransplantation Considered ConsideredAmiodarone Yes Yes Yes ConsideredPacemaker Yes (a Yes Considered

biventricular system may be preferred)

p46-52w23 2/13/04 11:20 AM 0


rals for cardiac transplantation (Kaye 1993). Patientswith intractable symptoms and end-stage diseasemay be referred for cardiac transplantation or forinsertion of a left ventricular assist device.

HCM In HCM the heart muscle is often not dilated– instead it is rather stiff with a normal pumpingcapacity. Beta-blockers, verapamil and diltazem areused to treat dyspnoea and chest pain, and toimprove exercise intolerance (McKenna and Behr2002). Diuretics may be used in the short term totreat pulmonary congestion. Arrhythmias are com-mon in patients with HCM, producing chest pain,syncope, dyspnoea and palpitations. Amiodaroneis an effective and widely used drug; however, itdoes have some unpleasant side effects such asskin sensitivity, sleep disturbances and thyroid prob-lems (McKenna and Behr 2002). Patients who exper-ience atrial fibrillation will require anticoagulationto reduce the risk of thrombus formation.

Some patients will have an obstructed blood flowfrom the left ventricle into the aorta, which mayincrease the symptoms of dyspnoea and syncope.Beta-blockers are the first-line treatment for obstruc-tion, with the majority of patients showing improve-ment in symptoms (McKenna and Behr 2002).Verapamil should be avoided in obstruction becauseof possible peripheral vasodilation and haemo-dynamic collapse (McKenna and Behr 2002).Disopyramide may be used to treat patients witha left ventricular outflow tract gradient, and it isbest used in combination, as used alone it mayaccelerate AV node conduction and increase thepotential risk of supraventricular arrhythmias (McKennaand Behr 2002).

For patients who become severely disabled dueto obstruction, there are three surgical options toconsider:� The ‘gold standard’ is septal myectomy – this

involves surgical widening of the outflow tract.The success rate is above 80 per cent, with amortality rate of less than 2 per cent and long-term symptom relief achieved in more than 70per cent of patients (McCully et al 1996).

� Another option is the use of a pacemaker. Thisoption remains controversial. The trials have onlyshown success on one in every three patientsand the results are not clearly reproducible.However, for some patients it achieves sympto-matic relief without the need for further surgi-cal intervention.

� The third option is alcohol ablation, which involvesthe injection of alcohol into the perforators ofthe left anterior descending coronary artery tocause a limited septal myocardial infarction (Spiritoet al 2000). This reduces septal hypertrophy andthe associated obstruction – with careful patientselection in experienced centres, alcohol abla-tion is very successful. Selected patients benefitfrom this technique provided they have suitablecoronary anatomy (Firoozi et al 2002).

ARVC Management is tailored to the individual’ssymptoms and the prevention of sudden cardiacdeath. Drug therapy is the first choice of treatmentfor patients with well-tolerated and non-life threat-ening ventricular arrhythmias. Beta-blockers andclass I and III anti-arrhythmic drugs are prescribed– sotalol or amiodarone (alone or in combinationwith beta-blockers) are the most effective drugswith a relatively low pro-arrhythmic risk (Corradoet al 1997). The efficacy of this treatment may bebased on reported symptoms and further evalua-tion using 24-hour holter and exercise testing. Inpatients with sustained ventricular tachycardia orventricular arrhythmia, anti-arrhythmic drug treat-ment guided by programmed ventricular stimula-tion with serial drug testing is an option (Corradoet al 1997). Sotalol has been reported to be themost effective anti-arrhythmic drug in the treat-ment of inducible and non-inducible VT in ARVC,with overall efficacy rates of more than 68 per centand 82 per cent respectively (Corrado et al 1997).However, its efficacy in preventing sudden deathremains uncertain. Patients who remain at high riskof sudden cardiac death are offered the option ofan ICD, which is the most effective safeguard againstarrhythmic sudden death and is the treatment ofchoice for survivors of cardiac arrest and thosepatients with VT (Corrado et al 2003). However,there may be complications associated with deviceimplantation due to pathologic changes in the rightventricular wall.

In patients in whom ARVC has progressed tosevere right ventricular or biventricular systolic dys-function with risk of thromboembolic complica-tions, treatment consists of current therapy for heartfailure, including diuretics, ACE inhibitors and dig-italis, as well as anticoagulants. Some patients mayrequire referral for heart transplantation.

ARVC is a progressive heart muscle disease thatmay present clinically different stages. Patients withARVC who have been evaluated and had treatmentinitiated should, with regular monitoring and treat-ment review, be able to lead a healthy life. Manyquestions remain unanswered in the diagnosis andmanagement of ARVC; further studies are in progressto prospectively evaluate the present diagnostic cri-teria and the natural history of the disease.RCM This is usually in its advanced stages beforeit is clinically recognised; the prognosis is poor witha two-year mortality of 35-50 per cent (Ammashet al 2000). The treatment of RCM is palliative and

Heart disorders

TIME OUT 6Sarah is found to have a left ventricularwall thickness of 15mm, a flat bloodpressure response on exercise andventricular tachycardia on holtermonitoring. She remains asymptomaticand feels physically well. What treatmentoptions does she have?

REFERENCESAmmash N et al (2000) Clinical profile

and outcome of idiopathic restrictive cardiomyopathy. Circulation. 101, 21,2490-2496.

Corrado D et al (2003) Implantablecardioverter-defibrillator therapy forprevention of sudden death in patientswith arrhythmogenic right ventricularcardiomyopathy/dysplasia. Circulation.108, 25, 3084-3091.

Corrado D et al (1997) Spectrum of clinicopathologic manifestations ofarrhythmogenic right ventricularcardiomyopathy/dysplasia: amulticenter study. Journal of theAmerican College of Cardiology. 30, 6,1512-1520.

Cox S et al (1997) Health-related qualityof life and psychological wellbeing inpatients with hypertrophiccardiomyopathy. Heart. 78, 2, 182-187.

Elliott P (2000) Dilated cardiomyopathy.Heart. 84, 1, 106-112.

Elliott P et al (2000) The prevention ofsudden death in hypertrophic cardiomyopathy. Expert Opinion onPharmacotherapy. 3, 5, 499-504.

Firoozi S et al (2002) Septal myotomy-myectomy and transcoronary septalalcohol ablation in hypertrophicobstructive cardiomyopathy. A comparison of clinical, haemodynamicand exercise outcomes. EuropeanHeart Journal. 23, 20, 1617-1624.

Fitzpatrick A et al (1990) Familial restrictivecardiomyopathy with atrioventricularblock and skeletal myopathy. BritishHeart Journal. 63, 2, 114-118.

Gilligan D et al (1991) Effects of a mealon haemodynamic function at rest andduring exercise in patients with hypertrophic cardiomyopathy. Journalof the American College of Cardiology.18, 2, 429-436.

Hamid M (2001) Arrhythmogenic rightventricular cardiomyopathy: diagnosisand management. InternationalJournal of Cardiology. 81, 8, 18-20.

Hesse A (1993) Cardiac amyloidosis: areview and report of a newtransthyretin (prealbumin) variant.British Heart Journal. 70, 2, 111-115.

Hirota Y (1990) Spectrum of restrictivecardiomyopathy: report of the nationalsurvey in Japan. American HeartJournal. 120, 1, 188-194.

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is similar to that of DCM and heart failure. Medicalintervention includes the use of diuretics, ACEinhibitors, anti-arrhythmic and anticoagulant drugs.A pacemaker may be used to treat AV conductionblock. Cardiac transplantation may be consideredin patients with refractory symptoms in idiopathicor familial RCM.

Newly diagnosed patients with cardiomyopathy areoften worried about genetic screening and inheri-tance. It is important that families are referred toappropriate genetic services. The nurse should be ableto provide basic genetic advice. Cardiomyopathycommonly has an autosomal dominant inheritancepattern, which means that only one copy of thedominant affected gene is required for the diseaseto be passed down to the offspring. Therefore eachchild has a 50 per cent chance of being affected,and males and females are affected equally (McKennaand Behr 2002).

Care of the patient with cardiomyopathy presentsthe cardiac nurse with many challenges. The pres-entation in a family may range from the asympto-matic patient who requires no treatment to suddencardiac death and heart failure. It is vital that adetailed family history is taken and recorded in theclinical notes, as often there is already a clear pic-ture of inheritance in a family. This information isessential for risk stratification and for identifyingother family members who may be at risk from thisdisease. Cardiomyopathy is a chronic condition withextensive emotional and social ramifications – anx-iety levels are high, especially in symptomatic patients(Cox et al 1997). Patients are confronted with apotentially life-threatening problem, and may per-ceive their levels of physical activity to be severelycurtailed, placing restrictions on everyday tasks.Cardiomyopathy is genetically inherited in manycases, giving rise to fear of transmission and issuessurrounding genetic counselling – parents oftenfeel responsible and guilty for their children’s con-dition. Many patients are diagnosed following asudden death in the family, so may already be suf-fering the pain of bereavement while attemptingto come to terms with their own health risk. Theimpact of the diagnosis is likely, therefore, to extend

beyond the immediate symptoms and limitationsof the condition.

It is clear that patients who receive detailed infor-mation on their condition, treatment and lifestyleare able to overcome these hurdles more easily (Coxet al 1997). These patients need the nurse to explaintheir condition, reassure them and offer them adviceabout prudent lifestyle changes. The CardiomyopathyAssociation provides patients with access to currentliterature on cardiomyopathy, as well as a networkof regional support. It is important to rememberthat you will encounter patients who are living withcardiomyopathy at various stages of disease progression– understanding the different types of cardio-myopathy, their inheritance patterns and treatmentstrategies will enable you to offer valuable supportand advice. Psychological adjustment to a diagno-sis of cardiomyopathy may depend on effectivecommunication with clinical staff, and support andunderstanding from family and friends (Steptoe etal 2000). The effect of chronic illness may producefeelings of fear, grief and loss that are essentiallyunending. Patients who are at risk of sudden deathalso have to live with a lack of predictability thatmakes adjustments to daily life more difficult.

Cardiomyopathy can have a distressing impact ona person’s health-related quality of life and emo-tional wellbeing. In this article the pathophysiology,clinical presentation and management of cardio-myopathy have been discussed. It is important toremember that you will encounter patients withcardiomyopathy at different stages of disease pro-gression, who will require various degrees of adviceand support. Understanding the different types ofcardiomyopathy will help you to evaluate the needsof individual patients and their families and carers.Providing patients with access to information abouttheir illness will help to dispel misconceptions andenable them to adapt their lifestyles to cope withtheir illness

Conclusion

Nursing considerations in cardiomyopathy

Heart disorders

TIME OUT 7It is clear from Sarah’s family history thatshe inherited hypertrophiccardiomyopathy from her father, as he has a similar pattern of hypertrophy.However, he remains well and symptom-free at the age of 66. Howwould you discuss the genetic inheritanceof HCM with this patient? Whatrecommendations would you give forscreening her three-year-old son and other siblings?

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Maron B et al (1995) Prevalence of hypertrophic cardiomyopathy in ageneral population of young adults;echocardiographic analysis of 4111subjects in the CARDIA Study.Circulation. 92, 4, 785-789.

McCully R et al (1996) Extent of clinicalimprovement after surgical treatmentof hypertrophic obstructive cardio-myopathy. Circulation. 94, 8, 467-471.

McKenna W et al (1981) Arrhythmia inhypertrophic cardiomyopathy: 1.Influence on prognosis. British HeartJournal. 46, 2, 168-172.

McKenna W, Behr E (2002) Hypertrophiccardiomyopathy: management, riskstratification, and prevention ofsudden death. Heart. 87, 2, 169-176.

Richardson P et al (1996) Report of the1995 World Health Organization/International Society and Federation ofCardiology Task Force on theDefinition and Classification ofCardiomyopathy. Circulation. 93, 5,841-842.

Sorajja P et al (2000) The moleculargenetics of hypertrophiccardiomyopathy: prognosticimplications. Europace. 2, 1, 4-14.

Spirito P et al (2000) Magnitude of leftventricular hypertrophy and risk ofsudden death in hypertrophiccardiomyopathy. New England Journalof Medicine. 342, 24, 1778-1785.

Steptoe A et al (2000) Health-relatedquality of life and psychological wellbeing in patients with dilatedcardiomyopathy. Heart. 83, 6, 645-650.

Sudhir S (1997) Restrictivecardiomyopathy. New England Journalof Medicine. 336, 4, 267-276.

The CardiomyopathyAssociation40 The Metro CentreTolpits LaneWatford, Herts WD1 8SBTel: 0800 018 1024www.cardiomyopathy.orgemail:[email protected]

The association is running aseries of educational seminarsaimed at health professionalsthroughout the UK oncardiomyopathy diagnosis,treatment and management.These seminars have RCNapproval and will be taught by aconsultant cardiologist and acardiomyopathy nurse specialist

Further information

TIME OUT 8Now that you have completed the article,you might like to write a practice profile.Guidelines to help you are on page 55.

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