Cardiomyopathy

Shenyang Medical College affiliated the second hospital

Zhang Xiao-Dan
 Definition
The cardiomyopathies are a
group of diseases that affect the
heart muscle itself and are not
the result of the major causes of
cardiac disease,i.e.,ischemic heart
disease, hypertension,valvular
disease, or congenital defects.
 Classification of CM
In many cases,however,it is not possible
to arrive at a specific etiologic diagnosis,and
thus it is often more desirable to classify the
cardiomyopathies into one of three types(di-
lated,hypertrophic,restrictive)on the basis of
differences in their pathophysiology and cli-
nical presentation.
 Dilated cardiomyopathy
 Dilated cardiomyopathies usually
present with symptoms and signs of
congestive heart failure(most commonly
dyspnea).Occasionally,symptomatic
ventricular arrhythmias are the presenting
event.
 Dilated cardiomyopathy
 Left and/or right ventricular systolic
pump function is impaired,leading to
progressive cardiac enlargement and hyper-
trophy,a process called remodeling.
Symptoms of CHF typically appear only
after remodeling has been ongoing for some
time (months or even years).
 Dilated cardiomyopathy
Pathogenesis of dilated cardiomyopathy
is thought to be due to one of the four
mechanisms:
(1)viral myocarditis
(2)familial disease
(3)immune abnormalities
(4)metabolic abnormalities
 Clinical manifestation
1. symptoms
(1) early: asymptom
(2) late: symptoms of left and right-
sided congestive failure,
manifested by dyspnea, peripheral
edema and palpitation,
hepatomegaly,thromboembolism,
sudden death.
 Clinical manifestation
In most patients,symptoms of
heart failure develop gradually.They
may be recognized because of
asymptomatic cardiomegaly or
electrocardiographic abnormalitis,
Including arrhythmias.The initial
presentation may be severe biven-
tricular failure.
 Clinical manifestation
Although vague chest pain may
be present,typical angina pectoris is
unusual and suggests the presence
of concomitant ischemic heart
disease. Syncope due to arrhythmia
and systemic embolism(often ema-
nating from a ventricular thrombus)
may occur.
 Clinical manifestation
2. Signs:
(1) heart enlarge
(2) diastolic 3rd sound gallop
(3) all kinds of arrhythmia
 Examination
1. X-ray: heart/chest >50%, ventricular
enlargement, pulmonary effusion
2. ECG: Arrhythmia such as atrial
fibrillation, block. ST-segment and T-
wave abnormalities
3. Echocardiography:
Left atrial and ventricular dilation
with normal or thinned walls and
systolic dysfunction, ejection
fraction reducing
 Examination

4. radionuclide scintigraphy
5.cardiac pipe inspection and
angiogram
6. endocardial biopsy
 Treatment
1. Drugs: the methods of therapy is to
control congestive heart failure and
arrhythmia.
2. Artifical cardiac pacing(cardiac
resynchronization,CRT) ,ICD(implan-
table cardioverter defibrillator) .
3. Surgical therapy, cardiac
transplantation
 Treatment
Few cases of cardiomyopathy are
amenable to specific therapy.Standard
therapy of heart failure with salt restric-
tion,angiotensin converting
enzyme(ACE)
inhibitors,diuretics,and digitalis
produces symptomatic improvement.
 Treatment
Most patients should be treated with
a β-adrenergic blocker.Spironolactone
should be added for most patients with
recent or current advanced heart failure.
 Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy(HCM)
Is characterized by left ventricular
hypertrophy,typically of a nondilated
chamber,without obvious cause such
as hypertension or aortic stenosis.
 Hypertrophic cardiomyopathy
Two features of HCM have attracted
the greatest attention:
(1)Asymmetric left ventricular hyper-
trophy,often with preferential hyper-
trophy of the interventricular septum.
 Hypertrophic cardiomyopathy
(2)A dynamic left ventricular outflow
tract pressure gradient,related to a
narrowing of the subaortic area as a
consequence of the midsystolic appo-
sition of the anterior mitral valve leaf-
let against the hypertrophied septum.
 Hypertrophic cardiomyopathy
The interventricular septum may be
disproportionately involved(asymme-
tric septal hypertrophy),but in some
cases the hypertrophy is localized to
the apex.
 Clinical manifestation
1. Symptoms: sudden death,
dyspnea, angina pectoris, fatigue,
syncope
2. Signs:
(1)slight cardiac enlargement
(2)the fourth sound
(3) systolic murmur
at the low left sternal border as
well as at the 3rd-4th intercostal
space. In cases with outflow
obstruction, a loud systolic murmur
is present that increases with upright
posture, nitroglycin ， positive
inotropic drugs or Valsalva’s
maneuver and decrease with
squatting , β- blockers.
Examination ：
1. X-ray: mild to moderate cardiac
enlargement
2. ECG: left ventricular hypertrophy, ST-T
abnormalities, abnormal Q waves
3. Echocardiogram:
(1) asymmetric septal hypertrophy (ASM)
(2) septal hypertrophy ≥ 1.3 thickness of
LV posterior wall
(3) systolic anterior motion (SAM)of the
mitral valve
 Treat ：
1. β- blockers, calcium antagonists
2. DDD artifical cardiac pacing: may
prevent the progression of
hypertrophy and obstruction.
3. Nonsurgical septal ablation has
been performed by injection of
alcohol into septal branches of
the left coronary artery.
4. Surgical therapy
 Restrictive cardiomyopathy
Restrictive cardiomyopathy is
characterized by impaired diastolic
filling with preserved contractile
function.
 Restrictive cardiomyopathy
Etiology:
This condition is relatively
uncommon,with the most frequent
causes being amyloidosis,radiation,
and myocardial fibrosis after open
heart surgery.
 Restrictive cardiomyopathy
Clinical manifestation:
The inability of the ventricle to fill
limits cardiac output and raises filling
pressure.Therefore,exercise intoleran-
ce and dyspnea are usually the most
prominent symptoms.
 Restrictive cardiomyopathy
Clinical manifestation:
The jugular venous pressure is
elevated and does not fall normally,or
it may rise with inspiration(Kussmaul's
sign).
 Restrictive cardiomyopathy
Laboratory Evaluation:
(1)ECG:conduction disturbances
low voltages
(2)Echocardiogram:thickened pericar-
dium
(3)Myocardial biopsies
(4)Surgical exploration:can make the
diagnosis.
 Restrictive cardiomyopathy
Treatment and prognosis:
Unfortunately,little useful therapy is
available for either the causative conditions
or restrictive cardiomyopathy itself.
Diuretics can help,but excessive diuresis
can produce worsening symptoms.Steroids
may be helpful in sarcoidosis but relieve
conduction abnormalities more often than
heart failure.
 Dilated Hypertrophic Restrictive
Frequent causes Idiopathic,alcoholic Hereditary Amyloidosis,post-
, syndrome,possibly radiation,post-open
myocarditis,post- chronic hyperten- heart surgery,dia-
partum,endocrino- sion betes,endomyocar-
pathies,genetic dial fibrosis
diseases
Symptoms Left or biventricular Dyspnea,chest pain Dyspnea,fatigue,
congestive heart syncope right-sided conges-
failure tive heart failure
Physical Cardiomegaly,S3, Sustained point of Elevated jugular
examination elevated jugular maximal impulse, venous pressure,
venous pressure, S4,variable systolic Kussmaul's sign
rales murmur,bisferiens
carotid pulse
ECG ST-T changes,con- Left ventricular ST-T changes,
duction abnormali- hypertrophy, conduction abnor-
ties,ventricular exaggerated malities,low voltage
ectopy septal Q wave
 Dllated Hypertrophic Restrictive

Chest X-ray Enlarged heart, Mild cardiomegaly Mild to moderate

pulmonary cardiomegaly
congestion
Echocardiogram, Left ventricular Left ventricular Small or normal left
nuclear studies dilation （ LV ＞ hypertrophy,asym- ventricular size,
60mm ） and metric septal hy- normal or mildly
dysfunction pertrophy,small left reduced left
ventricular size, ventricular function
normal or supra-
normal function,
systolic anterior
mitral motion,
diastolic
dysfunction

Cardiac Left ventricular Small,hypercontra- High diastolic pre-

catheterization dilation and dysfunc- ctile left ventricle, ssure,”square root”
tion,high diastolic dynamic outflow sign,normal or
pressures,low gradient,diastolic mildly reduced left
cardiac output dysfunction ventricular function
Pericardial Disease
 The pericardium stabilizes the
heart in anatomic position and
reduces contact between the heart
and surrounding structures.
 It is composed of fibrous tissure,
and although it will permit moderate
changes in cardiac size,it cannot
stretch rapidly enough to accomodate
rapid dilation of the heart or accumu-
lation of fluid without increasing intra-
pericardial(and,therefore,intracardiac)
pressure.
 Classification
Diseases of pericardium typically
present in one or more three clinical
forms:
(1)Acute pericarditis

(2)Pericardial effusion

(3)Pericardial constriction
 Classification
Progress classification:
(1)Acutepericarditis:less than 6 weeks
(2)Subacute pericarditis:6 weeks—6 months

(3)Chronic pericarditis:more than 6 months

 Acute pericarditis
Etiology
1. acute nonspecific pericarditis
2. infective pericarditis: virus, bacteria,
tuberculous and so on
3. autoimmunity: rheumapyra, connective
tissue disease(CTD), post heart damages
syndrome ( postinfarction syndrome)
 4. carcinomal pericarditis
5. metabolic disease: gout
6. physical cause: injure and radiation
7. diseases of near organ: AMI, aortic
dissection
 Pathology :
At early period of pericarditis,inflammation of
pericardium causes pericardial exudation with
fibrin,leucocyte and some times epithelial cells.
In that so called fibrinous pericarditis,patients will
have chest pain because of the rough friction be-
tween epicardial pericardium and parietal peri-
cardium.
 Pathology :
Pericardial friction rub may be heard at
site between the lower left sternal border
and the cardiac apex in a short period be-
fore fluid effusion.As pericarditis developed,
fluid accumulates in pericardial space and
pericardial effusion occurs.Then chest pain
may relieve and friction rub may alleviate or
even disappear.
 Clinical manifestation
1. Fibroproteinous pericarditis
(1) symptoms:
precardial pain: a sharp, dull
sensative radiate to neck and
shoulder, deep inspiration and
cough will precipitate the pain
(2) signs:
a pericarditis friction rub --- ---
characteristic sign
the third or fourth left intercostal
space
2. Pericardial Effusion
(1) symptoms:
dyspnea– outstanding symptom
(2) signs:
① the cardiac dullness↑
② the apical impulse weaken
or disappear
③ heart sound faint, far
④ Ewart’s sign
⑤ distension of jugular vein,
hepatomegaly, ascites
Ewart’s sign
A large effusion may increase
the area of cardiac dullness and
compress the lower lobe of the left
lung,causing atelectatic rales of
bronchial breath sounds and
egophony over the left lung base.
3.cardiac tamponade
(1) symptoms:tachycardia, distension
of jugular vein, venous pressure ↑
(2) signs:
① jugular venous distension ↑
② Bp ↓ SBp ↓ DBp(± )….shock
③ paradoxical pulse
④ signs of pericarditis effusion
3.cardiac tamponade

Clinical typical signs(Beck signs):

(1) 、 Bp ↓
(2) 、 heart sound faint
(3) 、 jugular venous distension
 Lab exam
1. Laboratory test: infective pericarditis–
WBC ↑ESR↑
2. X-ray: exudative pericarditis– heart
enlarge obviously without congestion of
lung --- ---This is a strong evidence of
pericarditis effusion.
3. ECG: acute stage :ST ↑, but not
including aVR
4. echocardiogram:
5. pericardiocentesis
main indications:
cardiac tamponade,
exudative pericarditis unknown cause
Diagnosis:
(1).Dyspnea
(2).Jugular venous distension
(3).Paradoxical pulse
(4).The cardiac dullness↑
(5).Heart sound faint, far
(6).Echocardiogram:pericardial effusion
 Constrictive pericarditis
Clinical manifestation
1. Symptoms: acute fatigue,
weakness, weigh loss, exertional
dyspnea, abdominal swelling,
ankle edema
2.signs: jugular vein distention,
hepatomegaly, ascites, heart sound↓ or
disappear, the cardiac dullness normal,
apical impulse↓↓,pericardial knock 50%
(+), HR irregular
accompanied by Af, SBp↓ ,kussmaul sign
(jugular venous distension↑ ↑ when
breathing)
lab exam:
1. X-ray: cardiac shape normal
pericardial calcification
2. ECG: low voltage
T wave inverse
3. Echocardiogram:
Typical manifestation of
echocardiogram:
(1).Thickening of pericardium
(2).Ventricular wall activity weaken
(3).Abnormal ventricular septal
movement
(4).Inferior vena cava broadening but
not change with breathe.
Treatment:
Initial treatment consists of gentle
diuresis.Surgical removal of the peri-
cardium,which should be complete,is
usually required in symptomatic pati-
ents but is associated with a relatively
high mortality rate.Operation should
be taken immediately after control of
pericardial infection or tuberculous
Pericarditis.
END
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