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E1=Cardiomyopathies are those diseases that primarily affect the heart muscle.

T
here are many ways of classifying the cardiomyopathies, each of which has its ow
n pros and cons. An anatomical description can be used to describe which ventri
cle is primarily affected. A haemodynamic description can be used to describe t
he effects of the cardiomyopathy upon the heart s function. Systolic heart failur
e is well recognised and results in impairment of the heart s contractile function
. Diastolic heart failure, which leads to stiffening and impaired filling of th
e ventricles during diastole, is more challenging to diagnose and its importance
and prevalence remain somewhat controversial. A temporal classification can be
used to identify patients who present acutely, often in pulmonary oedema, and p
atients who have a more chronic course to their disease. It should be remembere
d that heart failure is not a diagnosis in its own right and attempts should alw
ays be made to identify the underlying aetiology.
E2=The presentation of heart failure depends upon many factors, including the un
derlying aetiology and which ventricle is primarily affected. The patient s histo
ry will reflect this. Patients with left ventricular failure present predominan
tly with breathlessness on exertion and the severity of their symptoms can be cl
assified using the New York Heart Association Functional Classification. Patien
ts may also complain of breathlessness on lying flat and of waking during the ni
ght with breathlessness. Patients with right heart failure often present with p
eripheral oedema. As this becomes more severe they may also complain of abdomin
al distension and the associated symptoms of nausea and loss of appetite. It is
always essential to review the past medical history particularly when consideri
ng the underlying aetiology for the heart failure. Certain cardiomyopathies hav
e a hereditary component and so family history is also important. A drug histor
y is important to identify what medication the patient is already taking for the
ir problem and also what drugs, such as non-steroidal anti-inflammatory drugs, m
ay be contributing to the fluid retention.
E3=The examination findings in patients with chronic heart failure vary accordin
g to the underlying aetiology and which ventricle is primarily affected. In add
ition, many of the physical findings may be modified by treatment of patient. F
or instance, a patient optimally treated with diuretics may exhibit no signs of
fluid overload despite very poor cardiac function.
E4=Many conditions can mimic the clinical findings in heart failure and so makin
g a clinical diagnosis can be very difficult. In fact, several studies have sho
wn that fewer than half the patients with suspected heart failure actually have
demonstrable cardiac dysfunction on investigation. To make an accurate diagnosi
s, it is essential to obtain objective evidence of cardiac dysfunction. The Eur
opean Society of Cardiology therefore recommends that a diagnosis of heart failu
re should be based not just upon the presence of appropriate symptoms, but also
objective evidence of cardiac dysfunction at rest. If the diagnosis still remai
ns in doubt, then one should also look for an appropriate response to treatment
directed towards heart failure. There are many ways of obtaining objective evid
ence of cardiac dysfunction but the simplest and most readily available method i
s transthoracic echocardiography. Where practicable, it should always be perfor
med where heart failure is suspected. It is also good practice to perform the l
aboratory tests listed here, a 12 lead ECG and a chest X-ray. A great deal of i
nterest has been expressed in the use of brain natriuretic peptide as a screenin
g blood test for heart failure but this test has not yet been widely adopted. F
urther investigations that may be helpful to exclude myocardial ischaemia includ
e stress echocardiography, myocardial perfusion scanning and in selected patient
s, cardiac catheterization.
E5a=A 12 lead ECG has been recommended as a useful screening tool in patients wi
th suspected systolic heart failure, as it is usually abnormal. In fact, the pre
sence of a completely normal 12 lead ECG makes significant left ventricular syst
olic dysfunction very unlikely. The ECG shown here is from a patient with an isc
haemic cardiomyopathy and shows poor R wave progression across the chest leads.
E5b=A chest X-ray allows an estimate of heart size and will also identify pulmon
ary congestion or oedema. The first chest X-ray shows cardiomegaly with evidence
of upper lobe blood diversion in the lung fields. The second chest X-ray is fro
m another patient and has similar appearances. The third chest X-ray shows a lef
t ventricular aneurysm.
E5c=Echocardiography should be regarded as a standard investigation and allows n
ot just confirmation of ventricular dysfunction but also the identification of a
ssociated problems such as valvular disease. The first example is from a patient
with a dilated cardiomyopathy and left bundle branch block. Intraventricular co
nduction delays are seen in up to 30% of patient with heart failure and contribu
te to the haemodynamic impairment. The second example is from a patient with a l
arge myocardial infarction that has led to a large akinetic apical aneurysm.
E5d=Cardiac catheterisation also allows assessment of ventricular function and v
alvular disease. However it is particularly useful for the investigation of susp
ected coronary artery disease and for the identification of patients who may pot
entially benefit from revascularisation. This left ventriculogram shows globally
impaired left ventricular function.
E5e=Cardiac magnetic resonance imaging is not yet widely available but it is the
current gold standard technique for the measurement of cardiac volumes and func
tion. The example shown here shows a so-called non-compaction cardiomyopathy, as
evidenced by marked trabeculation of the left ventricle.
E5f=MUGA scanning is a nuclear technique of ventriculography and allows more acc
urate assessment of left ventricular ejection fraction than echocardiography.
E6=The non-pharmacological management of chronic heart failure is important. Pa
tients should receive advice on smoking cessation and should also moderate their
alcohol intake or, if alcohol has been a factor in their heart disease, stop dr
inking entirely. They should also reduce their salt intake and optimise their w
eight. Where available, exercise or cardiac rehabilitation programmes can prove
very useful. Patients should also be considered for pneumococcal immunisation
and an annual influenza immunisation. It is also important to identify exacerba
ting factors and drugs which may be contributing to the heart failure and deal w
ith them appropriately.
E7=Many drugs have been investigated for their value in the treatment of heart f
ailure. The mainstay drugs are listed here. Diuretics are important in symptom
atic patients in reducing fluid overload and maintaining the patient s dry weight.
ACE-inhibitors have not just symptomatic but also prognostic benefits and shou
ld be used routinely unless contraindicated. Contra-indications include renal a
rtery stenosis and hypotension. The available evidence suggests that the dose of
ACE-inhibitors should be gradually up-titrated as tolerated. Certain beta-bloc
kers have been shown to offer symptomatic and prognostic benefits and should be
used routinely unless patients have asthma or peripheral vascular disease. Such
beta-blockers, which include bisoprolol and carvedilol, should be introduced at
a very low dose and very slowly up-titrated over a period of many weeks. Patien
ts should be warned that they might feel worse before they feel better. Spirono
lactone has been demonstrated to have symptomatic and prognostic benefits in pat
ients with moderate to severe heart failure even if they are already receiving d
iuretics, ACE-inhibitors and beta-blockers. Digoxin has no effect on mortality
but has been shown to reduce episodes of hospitalisation for heart failure even
in patients in sinus rhythm. In the United Kingdom it is not used routinely but
is often reserved for those patients who remain symptomatic despite otherwise o
ptimal treatment.
E8=All the drugs listed here have demonstrable symptomatic benefits. The progno
stic benefits of diuretics are unproven as it would be difficult to perform an e
thical randomised trial. ACE-inhibitors and beta-blockers have proven prognosti
c benefits and should be used routinely where possible. Spironolactone also has
proven prognostic benefits in patients with moderate to severe heart failure an
d should be considered in this patient group. Digoxin has no effect on prognosi
s and is therefore usually reserved for patients who remain symptomatic despite
otherwise optimal treatment. Many of these drugs are potentially nephrotoxic and
so urea and electrolytes must be monitored closely.
E9=Invasive management of heart failure patients is sometimes appropriate. Pati
ents with ischaemic cardiomyopathy may gain some improvement in left ventricular
function following revascularisation. In this situation, dysfunctional myocardi
um is said to be hibernating. Hibernating myocardium can be demonstrated by a nu
mber of techniques including stress echocardiography, nuclear imaging and cardia
c magnetic resonance. Such investigations should therefore be considered in pati
ents with ischaemic cardiomyopathy. If myocardial hibernation is present they m
ay benefit from coronary angiography and subsequent revascularisation. Patients
in whom myocardial dysfunction is a consequence of scar tissue and in whom no h
ibernating myocardium can be identified, will not benefit from revascularisation
and may be exposed to unnecessary operative risks. Patients with valvular disea
se should be considered for surgical repair where the valvular disease has led t
o the heart failure. This can sometimes be a difficult assessment to make. Pat
ients with left ventricular dilatation often have a degree of functional mitral
regurgitation and, in the absence of any other obvious aetiology, it can sometim
es be challenging to identify cause and effect.
EX1=A lack of donor organs limits the availability of cardiac transplantation an
d around 25 to 30% of patients die whilst on the waiting list for transplant. In
patients below the age of 65 who have intractable heart failure despite optimal
medical management, cardiac transplantation can be considered. 90% of patients
referred for cardiac transplantation have either an ischaemic or dilated cardio
myopathy. Survival following a transplant is around 85% after 1 year and 70% af
ter 5 years.
EX2=The technique of biventricular pacing allows simultaneous pacing of the righ
t and left ventricles, restoring the ventricular synchrony that is lost in left
bundle branch block. In order to pace the left ventricle, a pacing lead has to b
e passed via the superior vena cava into the right atrium and then into the coro
nary sinus, a vein that runs around the atrioventricular groove at the back of t
he heart and drains into the right atrium. Positioning a pacing wire in the coro
nary sinus can be technically challenging. Once in the coronary sinus, the wire
is passed around the back of the heart until it lies behind the left ventricle.
E10=The presenting history of acute heart failure will usually be a fairly short
history of breathlessness, orthopnoea, and a cough with frothy sputum. However
this may occur on a background of chronic heart failure symptoms. The history
should also be used to identify why the heart failure has developed acutely. For
instance, is it secondary to an acute myocardial infarction, arrhythmia or is t
here any background cardiac problem that has suddenly changed? A relatively com
mon cause of acute symptoms, particularly on a background of chronic heart failu
re, is a change in medication. Physical examination may reveal some or all of t
he findings listed here.
E11=Echocardiography may show poor left ventricular function, and the presence o
f regional wall motion abnormalities would lead one to suspect myocardial ischae
mia or infarction. Echocardiography is also important to look for mechanical ca
uses as shown in the examples below.
E11a=The ECG in acute heart failure will help to identify acute myocardial ischa
emia or infarction, as shown here, or an underlying arrhythmia. This example sh
ows an acute anteroseptal ST-elevation myocardial infarction.
E11b=The chest X-ray will usually show signs of pulmonary congestion or oedema a
nd sometimes cardiomegaly in acute heart failure, as in the X-ray presented here
.
E11c=This patient with acute heart failure had a loud pansystolic murmur. The ec
hocardiogram showed a flail posterior mitral valve leaflet secondary to papillar
y muscle rupture. The head of the papillary muscle can be seen attached to the p
osterior mitral valve leaflet. Click on the second echo icon to see the severe m
itral regurgitation that precipitated the pulmonary oedema.
E11d=This echo shows a pseudoaneurysm of the inferior wall of the left ventricle
resulting from an inferior myocardial infarction.
E11e=This echo example shows a large ventricular septal defect resulting from a
septal myocardial infarction.
E12=A patient presenting with acute heart failure should be sat upright and give
n oxygen. Intravenous access should be obtained and the patient should be conne
cted to a cardiac monitor and pulse-oximeter. Intravenous opiates reduce anxiet
y and also help to reduce pre-load. Intravenous frusemide causes a diuresis but
also has a significant acute affect as a venodilator. Similarly, nitrates act
as venodilators and should be given as early as possible if blood pressure permi
ts. Beware of hypotension. The patient should be admitted to a coronary care u
nit as soon as practicable and any associated myocardial ischaemia or arrhythmia
s treated appropriately. Seek senior help if the patient fails to improve quick
ly and consider transferring to an intensive care unit. Inotropic support and v
entilation may be required. In the presence of myocardial ischaemia or infarcti
on, urgent coronary angiography may prove helpful in identifying a target lesion
for revascularisation. An intra-aortic balloon pump can be inserted at the sam
e time to reduce after-load and improve coronary perfusion. If a mechanical cau
se for the event is identified the patient should be referred for consideration
of urgent surgical correction of the problem.
E13=Cardiogenic shock is identified by the key features listed here. It has an
appalling prognosis, with over 80% of patients dying despite appropriate treatme
nt. The underlying cause should be identified and treated where possible. In th
e meantime the patient will often require support with oxygen and positive inotr
opes. Careful monitoring of fluid balance and intravascular volumes is essenti
al and a Swan Ganz catheter may prove helpful.
E14=An intra-aortic balloon pump is usually inserted via the femoral artery and
the balloon itself is positioned in the descending thoracic aorta just after the
origin of the left subclavian artery. The balloon inflates during diastole and
thereby helps improve coronary perfusion in ischaemia. The balloon then deflat
es during systole, reducing after-load and thereby enhancing forward flow. The b
alloon can be set to inflate during every diastolic interval, or during every se
cond or third interval, depending upon the level of support required. The video
shows the balloon pump support being reduced from 1:1 to 2:1.
E17=Acute myocarditis typically presents in a young and otherwise healthy patien
t. The median age at presentation is 42 years. Most patients have a history of
a recent viral illness and present with breathlessness and fatigue and often pa
lpitations in the presence of arrhythmias. Patients usually present in sinus ta
chycardia although atrial and ventricular arrhythmias are relatively common. Th
ere may be signs of cardiomegaly and the first heart sound may be soft. There m
ay be a gallop rhythm with a third or fourth heart sound present.
E18=The ECG in acute myocarditis often shows sinus tachycardia or other underlyi
ng arrhythmias. There are commonly non-specific ST segment and T wave abnormali
ties. The ECG shown here reveals sinus tachycardia and widespread T wave inversi
on. The chest X-ray may show cardiomegaly and evidence of pulmonary congestion o
r oedema as shown here. Cardiac enzymes and markers are elevated and it is prud
ent to check viral antibody titres, particularly when a flu-like prodrome is rep
orted. Echocardiography will identify the severity of left ventricular dysfuncti
on. In the example shown, left bundle branch block adds to the cardiac dysfuncti
on. Cardiac biopsy taken from the right ventricle is the gold standard for the d
iagnosis of acute myocarditis but does not need to be performed routinely unless
there is significant doubt about the diagnosis.
E19=Patients with acute myocarditis should be admitted for bed-rest and the moni
toring or treatment of any associated arrhythmias. If a treatable underlying ca
use can be identified, this should be managed accordingly. Supportive treatment
should be provided for symptoms and signs of heart failure. Although most pati
ents do improve with time, those who go on to develop a severe chronic cardiomyo
pathy may require consideration of cardiac transplantation.
E21=Dilated cardiomyopathy is the commonest of the cardiomyopathies. Some peo
ple use the term dilated cardiomyopathy only in relation to idiopathic cases. M
ore commonly, dilated cardiomyopathy is used to refer to cases where there is di
latation and impaired contraction of the left and/or right ventricles for which
there are many potential causes, some of which are listed here. It is also im
portant to remember that the different types of cardiomyopathy often overlap wit
h features of one or more types being present in an individual patient. Termino
logy is often used inconsistently and this can prove misleading. Ventricular dil
atation and impairment can also be an end result of many of the specific cardiom
yopathies such as ischaemic, hypertensive and valvular cardiomyopathy.
E22=The presenting history and examination findings in patients with dilated car
diomyopathy generally reflect the underlying aetiology and whether the disease p
rocess affects one or both ventricles. Patients typically present with a restin
g sinus tachycardia or may have developed atrial fibrillation. The combination
of a third heart sound and tachycardia creates a gallop rhythm. Pulmonary conge
stion and oedema may be evident, as may signs of right heart failure if there is
right ventricular involvement.
E23=The ECG will rarely be normal in dilated cardiomyopathy. There is often evi
dence of sinus tachycardia or atrial arrhythmias and other abnormalities may be
related to underlying heart disease such as previous infarction or hypertension.
A chest X-ray usually shows cardiomegaly and there may be evidence of pulmonary
congestion or oedema. The echocardiogram shows a dilated left and/or right ven
tricle with impaired systolic function. Cardiac catheterisation may be useful i
n ruling out an underlying ischaemic aetiology but cardiac biopsy is rarely nece
ssary.
E24=There is no specific treatment for cases of dilated cardiomyopathy although
any identifiable aetiology should be treated as appropriate. Treatment for hear
t failure and associated arrhythmias should be given as necessary. Cardiac trans
plantation may be appropriate in severe cases.
E26=Hypertrophic cardiomyopathy is about one fifth as common as dilated cardiomy
opathy. The key features are left and/or right ventricular hypertrophy, which i
s usually asymmetric and involves the interventricular septum. This septal hype
rtrophy often, but not always, causes obstruction to blood flow in the left vent
ricular outflow tract in which case the condition is referred to as hypertrophic
obstructive cardiomyopathy or HOCM. The tip of the anterior mitral valve leafle
t may be pulled towards the left ventricular outflow jet in systole. This moveme
nt is referred to as systolic anterior motion. This motion may cause failure of
coaptation with the posterior leaflet, giving rise to mitral regurgitation. Half
the cases are familial and usually show an autosomal dominant inheritance. The
re is a risk of sudden cardiac death in this condition and this may indeed be th
e presenting feature.
E27=Patients with hypertrophic cardiomyopathy may present with dizziness or sync
ope secondary to outflow obstruction or arrhythmias. Angina may occur even in t
he absence of coronary disease because of the increased oxygen demands of the hy
pertrophied myocardium. Palpitations and breathlessness may be present and sudd
en cardiac death may be the presenting feature. Asymptomatic cases are sometime
s detected on screening. Clinical examination may reveal a jerky carotid pulse
and a double apex beat that result from a palpable forceful atrial contraction s
econdary to atrial hypertrophy, which in itself is a consequence of the raised d
iastolic filling pressures of the left ventricle. This may also give rise to a
large A wave in the JVP. An ejection systolic murmur is audible if there is vent
ricular outflow obstruction. The murmur may be associated with a systolic thril
l. A pansystolic murmur of mitral regurgitation may be heard at the apex.
E28=The ECG usually shows evidence of left ventricular hypertrophy, as in the ex
ample shown here, often with T wave inversion. Ventricular ectopic beats or eve
n ventricular tachycardia may be evident. A chest X-ray is often normal but in
more advanced cases may show cardiomegaly and pulmonary congestion. Echocardiog
raphy allows identification of the hypertrophied myocardium and any associated o
utflow tract obstruction. The characteristic findings are asymmetric septal hyp
ertrophy, as shown in the first two examples, although this is not always the ca
se. Where there is left ventricular outflow tract obstruction, one often sees sy
stolic anterior motion of the anterior mitral valve leaflet, as seen in the thir
d echocardiogram here. 24 hour ECG monitoring is advisable to screen for ventri
cular arrhythmias. Cardiac catheterisation is not mandatory but helps identify
any associated coronary disease and permits further measurement of any outflow t
ract gradient. Cardiac magnetic resonance imaging can also be used to evaluate t
he ventricular anatomy as in the example of apical hypertrophic cardiomyopathy s
hown here.
E29=Beta-blockers are first line agents in patients with obstructive cardiomyopa
thy and symptoms. Beta-blockers improve diastolic filling by lengthening the du
ration of diastole and they also decrease myocardial oxygen consumption. Calciu
m channel blockers can also be useful either on their own or in combination with
a beta-blocker. Amiodarone is useful for both supraventricular and ventricular
arrhythmias where these have been documented. Vasodilators should be avoided i
n patients with outflow tract obstruction. Patients who remain symptomatic desp
ite appropriate drug therapy should be considered for dual chamber pacing which
reduces outflow obstruction. De-bulking of the hypertrophied septum can reduce
outflow obstruction and can be performed chemically, with injection of alcohol i
nto the first septal perforator branch of the left anterior descending artery vi
a a cardiac catheter, or by surgical de-bulking of the septum. Another importan
t aspect of patient management is family screening. All first-degree relatives
of a patient with hypertrophic cardiomyopathy should be advised to undergo scree
ning with a transthoracic echocardiogram.
E31=Restrictive cardiomyopathy results from stiffening of the myocardium that im
pairs filling of the ventricles during diastole. This usually results from infi
ltration or fibrosis of the ventricular myocardium. Conditions that can cause t
his are listed here. Although clinically restrictive cardiomyopathy can mimic c
onstrictive pericarditis, it is important to try and distinguish between these c
onditions since constrictive pericarditis can be treated surgically.
E32=The clinical features of restrictive cardiomyopathy are predominantly those
of breathlessness, fatigue and right heart failure. The JVP is elevated and the
X and Y descents are usually rapid. Kussmaul s sign, which is a paradoxical rise
in the JVP during inspiration, may be present but is also a feature of constric
tive pericarditis. There may be third and fourth heart sounds audible and the
carotid pulse is characteristically low volume. Signs of right heart failure in
clude peripheral oedema, hepatomegaly, and ascites. The patient should be exami
ned for any features suggestive of an underlying cause such as haemochromatosis
or scleroderma.
E33a=The ECG in restrictive cardiomyopathy often reveals atrial fibrillation and
relatively small QRS complexes. If the patient is still in sinus rhythm, evide
nce of P mitrale or P pulmonale may be seen. The example shown here is from a pa
tient in sinus rhythm with evidence of both right and left ventricular hypertrop
hy. The chest X-ray may reveal a normal heart size but with evidence of pulmonar
y congestion. The chest X-ray shown here reveals evidence of left ventricular hy
pertrophy.
E33b=The first set of echocardiograms show amyloid infiltration of the myocardiu
m. The ventricular myocardium is thickened and appears bright with impaired sys
tolic and diastolic function.
E33c=The second set of echocardiograms is from a patient with Loeffler s endocardi
tis in which the degranulation of eosinophils in the myocardium leads to fibrosi
s, particularly of the endocardium. A bright layer of fibrinous exudates can be
seen lining the endocardial walls. This layer is even more apparent on the seco
nd short axis view.
E33d=Cardiac catheterisation allows measurement of intracardiac pressures and th
eir relationship to respiration. This can be helpful in distinguishing restrict
ive cardiomyopathy from constrictive pericarditis. Cardiac biopsy can also be p
erformed.
E34=There is no specific treatment for restrictive cardiomyopathy. However when
it is due to either haemochromatosis or sarcoidosis then treatment of the under
lying condition may provide some benefit. Diuretics can be helpful in treating
associated heart failure, and any associated arrhythmias, most commonly atrial f
ibrillation, should be treated as appropriate. Beta-blockers and calcium channe
l blockers are likely to be unhelpful in restrictive cardiomyopathy and digoxin
is the agent of choice for the rate control of atrial fibrillation. Patients wi
th severe symptoms may be considered for cardiac transplantation.
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