Heart disorders

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Cardiomyopathy
In brief Author Stephanie Cruickshank RN, is Cardiomyopathy Nurse Specialist, The Heart Hospital, London. Email: stephanie.cruickshank@ uclh.org Summary Cardiomyopathy is a disease of the heart muscle. There are four main types of cardiomyopathy and each can affect people differently. This article discusses the signs and symptoms, diagnosis and treatment for the different types of the disease, the importance of genetic screening and risk stratification, and the nurses role in patient management. NS229 Cruickshank S (2004) Cardiomyopathy. Nursing Standard. 18, 23, 46-52. Date of acceptance: February 21 2003. Aim and intended learning outcomes The aim of this article is to provide an overview of the aetiology, treatment and clinical presentation of patients with cardiomyopathy and describe the role of the nurse in patient management. It also aims to highlight the psychosocial aspects that are a part of the disease and the ethical issues that arise in relation to familial evaluation and genetic testing. After reading this article you should be able to: I Recognise the difference between the types of cardiomyopathy. I Be aware of the prevalence and aetiology of the disease. I Realise the genetic inheritance of the condition and the importance of family screening. I Understand the mechanism and risk stratification for sudden death. I Identify current treatment trends for the various cardiomyopathies. I Describe the nurses role in the management of cardiomyopathy. Introduction Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction, and are classified as (Richardson et al 1996): I Dilated cardiomyopathy (DCM). I Hypertrophic cardiomyopathy (HCM). I Arrhythmogenic right ventricular cardiomyopathy (ARVC). I Restrictive cardiomyopathy (RCM). This article describes the various types of cardiomyopathies, their physiology and treatment, and discusses the psychological impact that the diagnosis of cardiomyopathy can bring information on patient support is also provided. Although there are many similarities with each type of cardiomyopathy, there are also distinct differences.

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

Different types of cardiomyopathy Dilated cardiomyopathy DCM is characterised by dilation and impaired contraction of the left ventricle (Figure 1) or both ventricles. Although in many cases the aetiology is not known, many factors appear to be associated with its development, for example, myocarditis, alcohol, metabolic disorders, infiltrative disorders, autoimmune disease, pregnancy and familial/genetic factors (Richardson et al 1996). It may also be associated with recognised cardiovascular disease in which the degree of myocardial dysfunction is not explained by the presenting pathology or the extent of ischaemic damage (Richardson et al 1996). Hypertrophic cardiomyopathy HCM is characterised by left and/or right ventricular hypertrophy, which is usually asymmetric and involves the interventricular septum (Figure 2). Typically, the left ventricular volume is normal or reduced, and is often associated with left ventricle outflow tract gradient. Arrhythmias and premature sudden death are common (Maron et al 1995). HCM is the most common cause of sudden death in people under the age of 30, and accounts for a high proportion of athlete-associated sudden deaths (Maron et al 1995). It occurs equally in both sexes and all races (Sorajja et al 2000). Although HCM is a chronic disease without a known cure, a number of treatments are available to alter its course. What causes HCM? HCM is a familial disease with an autosomal dominant inheritance pattern, which means that the condition may be passed from one generation to the next and does not skip a generation (McKenna and Behr 2002). The unexplained

Key words I Cardiovascular system and disorders I Heart disorders I Heart disorders: nursing These key words are based on subject headings from the British Nursing Index. This article has been subject to double-blind review.

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feature of the disease is that while some cases appear to affect the whole of the left ventricle, others may be confined to a small area of the ventricle. The disease expression in a family may vary from person to person, with some people severely affected and others less so. With the recent advances in genetics, gene carriers have been identified who do not have HCM but have the potential to pass it on to future generations. Arrhythmogenic right ventricular cardiomyopathy ARVC is a familial myocardial disease characterised pathologically by right ventricular (RV) myocardial atrophy and fibrofatty replacement, which means the heart muscle is gradually replaced by fatty tissue. Areas of fatty tissue may lead to weakness and bulges in the cardiac muscle wall (Figure 3). It is a progressive heart muscle disease that, with time, may lead to more diffuse RV involvement and left ventricular (LV) changes, and may culminate in heart failure (Hamid 2001). ARVC predominantly presents in adolescents or young adults, but it is increasingly being diagnosed in an older age group estimated figures of the incidence of ARVC range between 1:3,000 and 1:10,000 (Hamid 2001). It is a diagnosis that should be considered when presented with a patient who has ventricular tachycardia of left bundle branch morphology assessment is by means of a routine ECG. It may cause sudden cardiac death in a young person with what is considered to be a structurally normal heart (Hamid 2001). What causes ARVC? A familial background has been demonstrated in nearly 50 per cent of ARVC cases, with an autosomal dominant pattern of inheritance (Hamid 2001). As yet, the involved genes and the molecular defects remain unknown, but research continues in this area. In some patients there is either no evidence of inheritance or there is insufficient information about the individuals family to assess inheritance. A further difficulty is that in a family, features of the disease may be variable, and the disease may appear to skip a generation. Restrictive cardiomyopathy The fourth and least common type of cardiomyopathy is restrictive cardiomyopathy (RCM). RCM is defined as a heart muscle disease that results in impaired ventricular filling, with normal or decreased diastolic volume of either or both ventricles (Richardson et al 1996). Systolic function usually remains normal, at least early in the disease, and wall thickness may be normal or increased, depending on the underlying cause (Richardson et al 1996). The condition usually results from increased stiffness of the myocardium that causes pressure in the ventricle (or ventricles) to rise precipitously with only small increases in volume. As the condition affects either or both ventricles, the patient may present with signs of left or right ventricular failure. RCM may be a primary disorder due to endomyocardial fibrosis, Loefflers cardiomyopathy or Figure 1. Dilated heart Normal heart Pulmonary valve Mitral valve Dilated cardiomyopathy

RA

LA

LA RA

RV

LV

RV

LV

Tricuspid valve Aortic valve RA = right atrium RV = right ventricle LA = left atrium LV = left ventricle

Figure 2. Hypertrophic cardiomyopathy Asymmetric septal hypertrophy without obstruction

LA RA

Enlarged intraventricular septum

RV

LV

Reduced left ventricle

idiopathic cardiomyopathy (aetiology unknown) (Richardson et al 1996). Secondary RCM can be due to infiltrative disease such as amyloidosis, postirradiation therapy or storage diseases such as haemochromatosis, glycogen storage disease or Fabrys disease (Ammash et al 2000). Idiopathic RCM is sometimes familial and seems to be associated with skeletal myopathies, which may affect only the distal limbs (Fitzpatrick et al 1990). In childhood RCM may be more common in girls and has a poor prognosis compared with RCM in adults (Ammash et al 2000). february 18/vol18/no23/2004 nursing standard 47

Heart disorders
Figure 3. Arrhythmogenic right ventricular cardiomyopathy Fatty tissue may lead to weakness and bulges in the heart muscle wall accounts for a large number of patient referrals. Uncommonly, the patient may present with systemic embolism or sudden death (Elliott 2000). HCM Patients can present at any age with dyspnoea, chest pain, unexplained syncope, arrhythmia or sudden death (McKenna and Behr 2002). Many patients have no or only minor symptoms and in children and adolescents the diagnosis is often made during family screening. Exertional chest pain occurs in up to 30 per cent of adults, and many complain of atypical pain that is prolonged and occurs at rest and after meals (Gilligan et al 1991). Dyspnoea is common in adult patients, probably as a consequence of elevated pulmonary venous pressure resulting from impaired ventricular relaxation and filling (McKenna and Behr 2002). Patients less commonly present with symptoms such as waking up at night breathless and orthopnoeic, and require two or more pillows to sleep at night as a result. Approximately 15-25 per cent of patients experience syncope and 20 per cent complain of presyncope (Maron 1997). Palpitations are a frequent complaint (Maron 1997). ARVC The patient may be asymptomatic or may present with palpitations, syncope, presyncope, lethargy, dyspnoea and oedema (Hamid 2001). As with HCM the severity of symptoms and risk of complications vary greatly between people and many never experience any serious problems related to their condition. RCM The underlying cause of RCM may not be obvious on presentation the patient may present with symptoms similar to those of DCM. Exercise intolerance is a frequent symptom (Sudhir 1997). Uncommonly, the first presentation may be sudden cardiac death. Other symptoms include dyspnoea, paroxysmal nocturnal dyspnoea, orthopnoea, peripheral oedema, ascites, fatigue and weakness (Sudhir 1997). Angina does not occur except in amyloidosis, in which it may be the presenting symptom (Hesse 1993). In advanced cases, the patient may present with all the signs of heart failure except cardiomegaly. Up to one third of patients with idiopathic RCM may present with thromboembolic complications such as atrial fibrillation (Hirota 1990).

LA RA RA

LA

LV RV RV

LV

Fatty tissue Bulges in ventricular wall Dilation of right ventricle

Table 1. Signs and symptoms Type of cardiomyopathy Dilated Dyspnoea Syncope Sudden death Palpitations Chest pain Yes Yes Yes Yes Yes Hypertrophic Yes Yes Yes Yes Yes Arrhythmogenic Yes Yes Yes Yes Yes Restrictive Yes Yes Uncommon Yes Yes

Signs and symptoms The clinical signs and symptoms of all types of cardiomyopathy (Table 1) are established most easily and reliably with two dimensional echocardiography and ECG. In the presence of any abnormality further investigations may be required to secure the diagnosis and to plan treatment management. DCM Patients commonly present with signs and symptoms of heart failure, pulmonary congestion and/or low cardiac output, often with a background of exertional symptoms and fatigue for many months or years before their diagnosis (McKenna and Behr 2002). Symptoms may include dyspnoea, chest pain, embolic events such as atrial fibrillation, syncope and palpitations. An acute illness or the development of arrhythmia, in particular atrial fibrillation, may precipitate acute symptoms and prompt the individual to seek medical attention. Sometimes a patient is diagnosed as a result of family or routine medical screening, which is why it is important to take a careful family history, as family screening

TIME OUT 2
Sarah, a 26-year-old woman, presents to A&E following a cardiac arrest. After successful resuscitation she is diagnosed with hypertrophic cardiomyopathy. What investigations would be carried out to secure the diagnosis?

Diagnosis Investigations for cardiomyopathy are similar whichever type 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

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are usually performed to confirm the diagnosis. The patient may go on to have further tests, such as an exercise test, cardiac catheterisation and blood tests. HCM A detailed and accurate family history is important, especially noting any sudden unexplained deaths. The patient may be diagnosed as a result of family screening or as an incidental finding during a medical examination. Adults are often asymptomatic so in the majority of patients the physical examination may be unremarkable; however, in these asymptomatic patients, estimated mortality from HCM is still 1-2 per cent a year (Elliott et al 2000). Echocardiography may show a thickened left ventricle. The normal thickness is 12mm or less in HCM it may be 15mm or more (Elliott 2000 et al). It is important to establish the diagnosis early and assess the risk of sudden cardiac death so that the appropriate treatment can be given. Once a diagnosis of HCM has been made, it is vitally important that first-degree relatives of an affected person are offered cardiac evaluation. ARVC This might be difficult to diagnose as the patient may be asymptomatic until the first presentation with cardiac arrest. Also the diagnosis may be missed and the patient may present in later years with congestive heart failure with or without ventricular arrhythmia. Table 2. Investigations Type of cardiomyopathy Dilated Previous history Echocardiogram ECG Chest X-ray (to exclude other causes of breathlessness) Physical examination Exercise test Viral studies Endobiopsy 24-hour holter Magnetic resonance imaging/computed tomography (MRI/CT) Cardiac catheterisation Screening Signal average ECG
G G G G

Hypertrophic Arrhythmogenic
G G G G G G

Restrictive
G G G

Possibly

G G G G G

G G

G G

G G G

G G

G G G G G

Possibly
G G

Risk stratification for sudden death All patients should undergo non-invasive risk factor stratification with a clinical history, holter monitoring (24-hour continuous, portable ECG recording), and maximal exercise testing, regardless of symptomatic status or the apparent severity of morphological disease (McKeena and Behr 2002). HCM Sudden and unexpected death is the most devastating and unpredictable complication of HCM, but only a minority of patients are actually at risk, therefore it is important that all patients undergo non-invasive risk factor stratification with a clinical history, holter monitoring, and maximal exercise testing regardless of symptomatic status (McKenna and Behr 2002). Box 1 provides the risk factors that may lead to sudden death. Risk stratification, which identifies who is at high risk of sudden death, allows reassurance to be given to low-risk individuals, while high-risk patients can receive prophylactic intervention. Patients identified to be at high risk from sudden cardiac death should be offered prophylactic implantation of an implantable cardioverter defibrillator (ICD) and/or treatment with amiodarone (Box 2). An ICD is a device that uses similar technology to a pacemaker and is inserted under the skin in the chest area. If the patient develops a life-threatening arrhythmia, the device will recognise this and deliver a small electrical shock internally to restore normal rhythm. Patients who are at high risk should also be advised to avoid strenuous exercise or competitive sports, which require extreme physical exertion (McKenna and Behr 2002). Patients with restrictive cardiomyopathy may have undergone similar investigations; however with restrictive physiology the patient is more likely to progress to heart failure rather than sudden death. february 18/vol18/no23/2004 nursing standard 49

TIME OUT 3
How is cardiomyopathy inherited? Why is it important to take a detailed family history from the patient?

Genetic inheritance and family screening The inheritance pattern on HCM is the dominant trait, which means there is a 50 per cent chance of transmission to offspring. HCM Most patients with HCM have at least one other affected relative such as a parent or sibling (McKenna and Behr 2002). Therefore, it is important that all firstdegree relatives have a 12-lead ECG and an echocardiogram performed, as HCM may be present without causing symptoms. The expression of disease is agerelated, occurring during or soon after periods of rapid growth. Cardiovascular abnormalities are commonly detected during adolescence and children of an affected individual should undergo cardiac evaluation at six-monthly intervals until they reach adulthood; thereafter if the disease is late-onset evaluation should be performed every five years. Genetic testing is only available at research centres, but exciting progress has been made and it is hoped that, in the future, genetic evaluation will be available to all.

TIME OUT 4
What risk factors are associated with sudden death in HCM? Which investigations should be carried out to assess this risk?

Heart disorders
Table 3. Treatments used in cardiomyopathy Type of cardiomyopathy Dilated Angiotensinconverting enzyme (ACE) inhibitors All antagonists Diuretics Surgery Beta-blockers Implantable cardioverter defibrillator (ICD) insertion Transplantation Amiodarone Pacemaker Yes Hypertrophic Arrhythmogenic Restrictive Considered Yes Box 2. Implantable cardioverter defibrillator Implantable cardioverter defibrillators (ICDs) have been proven to prolong survival in high-risk cardiac patients. An ICD is a relatively small device that is implanted under the skin, most commonly in the upper chest. It consists of a battery (lasting three-six years), energy delivery components and electronic circuitry, which are all sealed in one case. The ICD is connected to the heart via one or more electrodes that are inserted into veins. Through these electrodes, the ICD monitors the patients heart rate and rhythm and can deliver corrective electrical treatment as appropriate. The ICD can deliver high-energy therapy (shocks) to correct serious life-threatening rapid and sustained arrhythmias. The patient may feel this therapy as a kick in the chest or he or she may lose consciousness before the shock is delivered. In some patients the ICD is used in conjunction with anti-arrhythmic drugs.

Yes Yes Yes Considered Yes Yes Considered

Considered Considered Yes Considered

Yes Yes Yes

Considered Yes Yes (a biventricular system may be preferred)

Yes Yes

Yes Considered

Considered Considered

Box 1. Recognised markers of increased risk of sudden death in HCM I Previous cardiac arrest I Non-sustained ventricular tachycardia on holter or exercise I Abnormal exertional blood pressure response I Unexplained syncope I Family history of premature sudden death I Severe left ventricular hypertrophy >30mm (McKenna and Behr 2002)

ARVC Risk stratification is an important part of patient management with the intention of preventing arrhythmic sudden death. Important clinical markers include a history of syncope, a malignant family history, diffuse right ventricular dilation with a reduction in ejection fraction and patients with left ventricular involvement (McKenna and Behr 2002). Sudden death may be the first manifestation of the disease, mostly in previously asymptomatic young adults and athletes.

TIME OUT 5
Sarah develops hyperthyroidism as a result of amiodarone therapy. The option of an ICD is discussed. What are the common fears of patients receiving an ICD? How might you help her to overcome these?

Treatment options A fundamental goal of treatment in cardiomyopathy is the alleviation of symptoms and the prevention of sudden cardiac death (Table 3). DCM This disease has many causes and it is important that it is detected in the early stages so that the patient can receive the most appropriate therapy. Recent advances in understanding the pathophysiology of the disease and treatments have substantially improved the outlook for many patients. The primary aim of treatment is to control symptoms and to prevent disease progression and complications, 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 irrespective of the severity of heart failure. These drugs improve dyspnoea and exercise tolerance, and reduce hospitalisation rates and cardiovascular mortality (Elliott 2000). They have also been shown to prevent or slow down disease progression in asymptomatic patients (Elliott 2000). Angiotensin II receptor antagonists may be prescribed in the case of intolerable side effects from ACE therapy, as they have a similar therapeutic effect (Elliott 2000). One of the major concerns for patients with DCM is the risk of sudden death. Recent work has demonstrated substantial reductions in sudden death and death from progressive heart failure in patients treated with beta-blockers (Elliott 2000) the initial dose should be low and careful monitoring and titration is required. Another drug used is spironolactone, which has been shown to reduce the risk of sudden death by 30 per cent in patients with symptoms of moderate to severe heart failure (Elliott 2000). Anticoagulation may be required in patients with dilated atrial chambers and those with atrial fibrillation. Care should be taken when prescribing anti-arrhythmic drugs in DCM, as some may be pro-arrhythmic, that is, they may precipitate arrhythmias. Multisite ventricular pacing Dual chamber pacing has been advocated as a method for restoring atrium/ ventricle (AV) synchrony and improving left ventricular co-ordination in patients with severe congestive heart failure. Biventricular pacing has been used with success, especially in patients with a QRS greater than 150ms and a prolonged PR interval, and symptoms refractory to conventional medical treatment (Elliott 2000). Patients who are at high risk of sudden death may require an ICD to restore normal heart rhythm (Box 2). DCM accounts for up to 50 per cent of refer-

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rals for cardiac transplantation (Kaye 1993). Patients with intractable symptoms and end-stage disease may be referred for cardiac transplantation or for insertion of a left ventricular assist device. I The third option is alcohol ablation, which involves the injection of alcohol into the perforators of the left anterior descending coronary artery to cause a limited septal myocardial infarction (Spirito et al 2000). This reduces septal hypertrophy and the associated obstruction with careful patient selection in experienced centres, alcohol ablation is very successful. Selected patients benefit from this technique provided they have suitable coronary anatomy (Firoozi et al 2002). ARVC Management is tailored to the individuals symptoms and the prevention of sudden cardiac death. Drug therapy is the first choice of treatment for patients with well-tolerated and non-life threatening ventricular arrhythmias. Beta-blockers and class I and III anti-arrhythmic drugs are prescribed sotalol or amiodarone (alone or in combination with beta-blockers) are the most effective drugs with a relatively low pro-arrhythmic risk (Corrado et al 1997). The efficacy of this treatment may be based on reported symptoms and further evaluation using 24-hour holter and exercise testing. In patients with sustained ventricular tachycardia or ventricular arrhythmia, anti-arrhythmic drug treatment guided by programmed ventricular stimulation with serial drug testing is an option (Corrado et al 1997). Sotalol has been reported to be the most effective anti-arrhythmic drug in the treatment of inducible and non-inducible VT in ARVC, with overall efficacy rates of more than 68 per cent and 82 per cent respectively (Corrado et al 1997). However, its efficacy in preventing sudden death remains uncertain. Patients who remain at high risk of sudden cardiac death are offered the option of an ICD, which is the most effective safeguard against arrhythmic sudden death and is the treatment of choice for survivors of cardiac arrest and those patients with VT (Corrado et al 2003). However, there may be complications associated with device implantation due to pathologic changes in the right ventricular wall. In patients in whom ARVC has progressed to severe right ventricular or biventricular systolic dysfunction with risk of thromboembolic complications, treatment consists of current therapy for heart failure, including diuretics, ACE inhibitors and digitalis, as well as anticoagulants. Some patients may require referral for heart transplantation. ARVC is a progressive heart muscle disease that may present clinically different stages. Patients with ARVC who have been evaluated and had treatment initiated should, with regular monitoring and treatment review, be able to lead a healthy life. Many questions remain unanswered in the diagnosis and management of ARVC; further studies are in progress to prospectively evaluate the present diagnostic criteria and the natural history of the disease. RCM This is usually in its advanced stages before it is clinically recognised; the prognosis is poor with a two-year mortality of 35-50 per cent (Ammash et al 2000). The treatment of RCM is palliative and

TIME OUT 6
Sarah is found to have a left ventricular wall thickness of 15mm, a flat blood pressure response on exercise and ventricular tachycardia on holter monitoring. She remains asymptomatic and feels physically well. What treatment options does she have?

HCM In HCM the heart muscle is often not dilated instead it is rather stiff with a normal pumping capacity. Beta-blockers, verapamil and diltazem are used to treat dyspnoea and chest pain, and to improve exercise intolerance (McKenna and Behr 2002). Diuretics may be used in the short term to treat pulmonary congestion. Arrhythmias are common in patients with HCM, producing chest pain, syncope, dyspnoea and palpitations. Amiodarone is an effective and widely used drug; however, it does have some unpleasant side effects such as skin sensitivity, sleep disturbances and thyroid problems (McKenna and Behr 2002). Patients who experience atrial fibrillation will require anticoagulation to reduce the risk of thrombus formation. Some patients will have an obstructed blood flow from the left ventricle into the aorta, which may increase the symptoms of dyspnoea and syncope. Beta-blockers are the first-line treatment for obstruction, with the majority of patients showing improvement in symptoms (McKenna and Behr 2002). Verapamil should be avoided in obstruction because of possible peripheral vasodilation and haemodynamic collapse (McKenna and Behr 2002). Disopyramide may be used to treat patients with a left ventricular outflow tract gradient, and it is best used in combination, as used alone it may accelerate AV node conduction and increase the potential risk of supraventricular arrhythmias (McKenna and Behr 2002). For patients who become severely disabled due to obstruction, there are three surgical options to consider: I The gold standard is septal myectomy this involves surgical widening of the outflow tract. The success rate is above 80 per cent, with a mortality rate of less than 2 per cent and longterm symptom relief achieved in more than 70 per cent of patients (McCully et al 1996). I Another option is the use of a pacemaker. This option remains controversial. The trials have only shown success on one in every three patients and the results are not clearly reproducible. However, for some patients it achieves symptomatic relief without the need for further surgical intervention.

REFERENCES Ammash N et al (2000) Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation. 101, 21, 2490-2496. Corrado D et al (2003) Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation. 108, 25, 3084-3091. Corrado D et al (1997) Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. Journal of the American College of Cardiology. 30, 6, 1512-1520. Cox S et al (1997) Health-related quality of life and psychological wellbeing in patients with hypertrophic cardiomyopathy. Heart. 78, 2, 182-187. Elliott P (2000) Dilated cardiomyopathy. Heart. 84, 1, 106-112. Elliott P et al (2000) The prevention of sudden death in hypertrophic cardiomyopathy. Expert Opinion on Pharmacotherapy. 3, 5, 499-504. Firoozi S et al (2002) Septal myotomymyectomy and transcoronary septal alcohol ablation in hypertrophic obstructive cardiomyopathy. A comparison of clinical, haemodynamic and exercise outcomes. European Heart Journal. 23, 20, 1617-1624. Fitzpatrick A et al (1990) Familial restrictive cardiomyopathy with atrioventricular block and skeletal myopathy. British Heart Journal. 63, 2, 114-118. Gilligan D et al (1991) Effects of a meal on haemodynamic function at rest and during exercise in patients with hypertrophic cardiomyopathy. Journal of the American College of Cardiology. 18, 2, 429-436. Hamid M (2001) Arrhythmogenic right ventricular cardiomyopathy: diagnosis and management. International Journal of Cardiology. 81, 8, 18-20. Hesse A (1993) Cardiac amyloidosis: a review and report of a new transthyretin (prealbumin) variant. British Heart Journal. 70, 2, 111-115. Hirota Y (1990) Spectrum of restrictive cardiomyopathy: report of the national survey in Japan. American Heart Journal. 120, 1, 188-194.

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Kaye M (1993) The registry of international society for heart and lung transplantation. Tenth official report. Journal of Heart and Lung Transplant. 12, 4, 541-548. Maron B (1997) Hypertrophic cardiomyopathy. Lancet. 350, 9071, 127-133. Maron B et al (1995) Prevalence of hypertrophic cardiomyopathy in a general population of young adults; echocardiographic analysis of 4111 subjects in the CARDIA Study. Circulation. 92, 4, 785-789. McCully R et al (1996) Extent of clinical improvement after surgical treatment of hypertrophic obstructive cardiomyopathy. Circulation. 94, 8, 467-471. McKenna W et al (1981) Arrhythmia in hypertrophic cardiomyopathy: 1. Influence on prognosis. British Heart Journal. 46, 2, 168-172. McKenna W, Behr E (2002) Hypertrophic cardiomyopathy: management, risk stratification, and prevention of sudden death. Heart. 87, 2, 169-176. Richardson P et al (1996) Report of the 1995 World Health Organization/ International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathy. Circulation. 93, 5, 841-842. Sorajja P et al (2000) The molecular genetics of hypertrophic cardiomyopathy: prognostic implications. Europace. 2, 1, 4-14. Spirito P et al (2000) Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. New England Journal of Medicine. 342, 24, 1778-1785. Steptoe A et al (2000) Health-related quality of life and psychological wellbeing in patients with dilated cardiomyopathy. Heart. 83, 6, 645-650. Sudhir S (1997) Restrictive cardiomyopathy. New England Journal of Medicine. 336, 4, 267-276.

is similar to that of DCM and heart failure. Medical intervention includes the use of diuretics, ACE inhibitors, anti-arrhythmic and anticoagulant drugs. A pacemaker may be used to treat AV conduction block. Cardiac transplantation may be considered in patients with refractory symptoms in idiopathic or familial RCM.

TIME OUT 7
It is clear from Sarahs family history that she inherited hypertrophic cardiomyopathy from her father, as he has a similar pattern of hypertrophy. However, he remains well and symptom-free at the age of 66. How would you discuss the genetic inheritance of HCM with this patient? What recommendations would you give for screening her three-year-old son and other siblings?

Nursing considerations in cardiomyopathy Newly diagnosed patients with cardiomyopathy are often worried about genetic screening and inheritance. It is important that families are referred to appropriate genetic services. The nurse should be able to provide basic genetic advice. Cardiomyopathy commonly has an autosomal dominant inheritance pattern, which means that only one copy of the dominant affected gene is required for the disease to be passed down to the offspring. Therefore each child has a 50 per cent chance of being affected, and males and females are affected equally (McKenna and Behr 2002). Care of the patient with cardiomyopathy presents the cardiac nurse with many challenges. The presentation in a family may range from the asymptomatic patient who requires no treatment to sudden cardiac death and heart failure. It is vital that a detailed family history is taken and recorded in the clinical notes, as often there is already a clear picture of inheritance in a family. This information is essential for risk stratification and for identifying other family members who may be at risk from this disease. Cardiomyopathy is a chronic condition with extensive emotional and social ramifications anxiety levels are high, especially in symptomatic patients (Cox et al 1997). Patients are confronted with a potentially life-threatening problem, and may perceive their levels of physical activity to be severely curtailed, placing restrictions on everyday tasks. Cardiomyopathy is genetically inherited in many cases, giving rise to fear of transmission and issues surrounding genetic counselling parents often feel responsible and guilty for their childrens condition. Many patients are diagnosed following a sudden death in the family, so may already be suffering the pain of bereavement while attempting to come to terms with their own health risk. The impact of the diagnosis is likely, therefore, to extend

beyond the immediate symptoms and limitations of the condition. It is clear that patients who receive detailed information on their condition, treatment and lifestyle are able to overcome these hurdles more easily (Cox et al 1997). These patients need the nurse to explain their condition, reassure them and offer them advice about prudent lifestyle changes. The Cardiomyopathy Association provides patients with access to current literature on cardiomyopathy, as well as a network of regional support. It is important to remember that you will encounter patients who are living with cardiomyopathy at various stages of disease progression understanding the different types of cardiomyopathy, their inheritance patterns and treatment strategies will enable you to offer valuable support and advice. Psychological adjustment to a diagnosis of cardiomyopathy may depend on effective communication with clinical staff, and support and understanding from family and friends (Steptoe et al 2000). The effect of chronic illness may produce feelings of fear, grief and loss that are essentially unending. Patients who are at risk of sudden death also have to live with a lack of predictability that makes adjustments to daily life more difficult. Conclusion Cardiomyopathy can have a distressing impact on a persons health-related quality of life and emotional wellbeing. In this article the pathophysiology, clinical presentation and management of cardiomyopathy have been discussed. It is important to remember that you will encounter patients with cardiomyopathy at different stages of disease progression, who will require various degrees of advice and support. Understanding the different types of cardiomyopathy will help you to evaluate the needs of individual patients and their families and carers. Providing patients with access to information about their illness will help to dispel misconceptions and enable them to adapt their lifestyles to cope with their illness

Further information The Cardiomyopathy Association 40 The Metro Centre Tolpits Lane Watford, Herts WD1 8SB Tel: 0800 018 1024 www.cardiomyopathy.org email:cmaassoc@aol.com The association is running a series of educational seminars aimed at health professionals throughout the UK on cardiomyopathy diagnosis, treatment and management. These seminars have RCN approval and will be taught by a consultant cardiologist and a cardiomyopathy nurse specialist

TIME OUT 8
Now 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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