Transcript Cardiomyopathies

Nonrheumatic carditis in children.
Cardiomyopathy.
Prof. Pavlyshyn H.A.
Nonrheumatic carditis
• is the group of heterospecific inflammatory cardiac diseases
with the affection of myocardium, but there is possibility of
spreading of process on all its shells,
• are caused by an infection, due to direct invasion in
myocardium or toxins or autoantigens on it.
• are present in all age-related groups, but more frequent in children of the
first years of life with predominance of boys;
• From data of autopsy nonrheumatic carditises are finding in 3-9% of
children, died from different reasons
Nonrheumatic carditis
• refers to inflammation and may be caused by many
infections, toxic (cocaine, ethanol, heavy metals) or
idiopathic processes (giant cell myocarditis, diabetes
mellitus, sarcoidosis, systemic lupus erythematosus,
thyrotoxicosis), affecting the myocardium with or
without associated systemic manifestations of disease
process or involvement of the endocardium or
pericardium;
• The most common manifestation is heart failure,
although arrhythmias and sudden death may be the 1st
detectable signs.
Nonrheumatic carditis
Its manifestations are age dependent:
• in early infancy- viral myocarditis often occurs as a
acute, fulminant disease;
• in toddlers, young children - it occurs as an acute
myopericarditis;
• in older children, adolescents – it is often
asymptomatic and comes to clinical attention
primarily as a precursor to IDCM.
ETIOLOGY
• Viruses: enteroviruses - ECHO, coxsackievirus B;
adenoviruses, influenza, parainfluenza, Epstein-Bar,
poliomyelitis, measles, chicken-pox etc.
• Bacteria (Diptheria, N. meningitidis, M. pneumonia, betahemolytic strep),
• Fungi – aspergillus, candida
• Alergic reactions on vaccination, food, domestic, medicinal
allergens (Penicillin, Sulfanilamide)
• Toxic influences (diphtheria, botulism)
• Physical agents, radiation
• Indefinite factors (idiopathic)
Pathophysiology
• Myocarditis is characterized by myocardial inflammation, injury or
necrosis, and ultimately fibrosis.
• Cardiac enlargement and diminished systolic function occur as a
direct result of the myocardial damage.
• Viral myocarditis may also become a chronic process with
persistence of viral nucleic acid in the myocardium, and the
perpetuation of chronic inflammation secondary to altered host
immune response including activated T lymphocytes and antibodydependent cell mediated damage.
• Cytokines such as tumor necrosis factor-α and interleukin-1 are
inhibitors of myocyte response to adrenergic stimuli and result in
diminished cardiac function.
• The final result of viral-associated inflammation can be dilated
cardiomyopathy.
PATHOGENESIS OF CARDITIS
Influence of etiologic factor on cardiomyocytes

Production of inflammatory mediators
•

Destruction of affected cells
•

Inflammation in myocardium is spread on endo- pericardium
•
•
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Immunopatologic reaction of slow type with formation of immune complexes

Hypercoagulation – disorders of microcirculation
•

Sclerosis of cardiomyocytes
•
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Arrhythmias, cardiac failure
CLASSIFICATION OF NONRHEUMATIC CARDITISES
 According to the time of origin:
congenital (early, late), acquired
 Etiology: viral, bacterial, allergic, toxic,
idiopathic
 Severity: mild, moderate, severe
 The form: acute (up to 3 mo), subacute (to 12 mo); chronic
(more than 12mo), primary-chronic
Activity of process: active - І, ІІ, ІІІ degree; nonactive
 Stage of blood circulation failure: І; ІІ A, B; ІІІ
CRITERIA OF NONRHEUMATIC CARDITISES
AT CHILDREN, ACCORDING TO NEW-YORKS'
CARDIOLOGY ASSOCIATION
•
•
•
•
presence of proofs of the clinical or laboratory infection
clinical and instrumental cardiomegaly
weak І tone
changes of rhythm - tachy- bradycardia, violation of
rhythm, gallop rhythm
• pathological changes ECG (violations of rhythm or
processes of repolarisation in myocardium)
• increase of LDG1 / LDG2 level
• congestive cardiac failure
Presence of 3 or more signs testifies to the credible
diagnosis of carditis
Clinical manifestations
• Manifestations of myocarditis range from asymptomatic or
nonspecific illness to acute cardiogenic shock and sudden death.
• Infants and young children more often have a fulminant
presentation with fever, respiratory distress, tachycardia,
hypotension, gallop rhythm and cardiac murmur. Associated
findings may include a rash or signs of hepatitis, aseptic
meningitis.
• Patients with acute or chronic myocarditis may present
with chest discomfort, fever, palpitations, easy fatigability, or
syncope. Hepatic enlargement, peripheral edema, and
pulmonary findings such as wheezes or rales may be present in
patients with decompensated congestive heart failure.
Clinical manifestations
• Arrhythmias may be the first clinical manifestation;
• Cyanosis, skin discolouration in blue or grey tones;
• Swelling in the face, feet or legs; poor circulation, showing as
cold hands and feet;
• Severe heart failure - distant heart sounds, weak pulses,
tachycardia out of proportion to the fever, mitral insufficiency
caused by dilatation of the valve annulus, gallop rhythm,
respiratory distress, acidosis, shock.
• In the most fulminate form, death may occur within 1-7 days of
the onset of symptoms.
DIAGNOSIS
• ESR, heart enzymes (creatine phosphokinase, lactate dehydrogenase),
BNP (brain natriuretic peptide), cardiac troponin may be elevated;
• PCR of serum samples and endomyocardial biopsy have
shown viral genome and identifying inflammatory cell infiltrates
or myocyte damage;
• X-ray chest – enlarged heart, pulmonary edema;
• Echo-CG – poor ventricular function, often pericardial effusion,
mitral valve regurgitation.
• Cardiac catheterization and endomyocardial biopsy – can
detect other causes of cardiomyopathy (mitochondrial defects, storage
disease).
ECG – tachycardia, reduced QRS complex voltage,
ST-segment and T-wave abnormalities
Paroxismal
tachycardia
Premature ventricular
beats
Endocardial fibroelastosis – EFE
fetal endocarditis, endocardial fibrosis,
prenatal fibroelastosis
In primary EFE
– the left ventricular
chamber is dilated
In secondary EFE
- the ventricular cavity
is often contracted
In secondary EFE, severe congenital
heart disease of the left-side obstructive
type (AS, hypoplastic left heart syndrom,
severe CoA) is present.
Endocardial fibroelastosis – EFE
fetal endocarditis, endocardial fibrosis,
prenatal fibroelastosis
Clinical manifestations
• Dyspnea, cough, anorexia, hepatomegaly, edema, failure to thrive,
recurrent pulmonary infections.
• X-ray chest – cardiac enlargement
• ECG – signs of LA, LV hypertrophy with strain
• Echo-CG – dilated, poorly functioning LV.
• MRI – may delineate the fibrotic endomyocardial surface.
• Infants in whom valvular lesions or associated congenital
cardiovascular defects are predominant die in the 1st mo of life.
Endocardial fibroelastosis – EFE
Roentgenograms confirm
significant cardiac enlargement;
Note the enlargement of the heart,
without a distinctive contour and
clear lung fields
Endocardial fibroelastosis
ACUTE CARDITIS
.
 develops directly at the time of viral infection or soon after such
infection.
 The severe forms are observed predominantly at the age below
3 years.
 The forms of the moderate severity may be observed both in the
children of early age and in preschool and school age children.
 The mild forms are recorded predominantly in preschool and
school age children
ACUTE CARDITIS
• Pale skin, weakness, headache, decline of appetite, fever,
shortness of breath;
• for elder children - cardialgia, heartbeating;
• Chest pain due to coexisting pericarditis
• Enlarged heart, tachycardia, weakness of heart tones, soft
systolic murmur on the apex, decreased arterial pressure
• ECG: decrease of waves voltage, conduction impairments,
arrhythmias
• Echo-CG: myocardium is diffuse thick, dilatation of chambers,
reduced myocardial contractility, possible presence of liquid in
pericardium
• Increased levels of LDG1, LDG2, CPK (creatine
phosphokinase), BNP (brain natriuretic peptide)
SUBACUTE CARDITIS
Occurs through 3-4 mos after acute carditis or is diagnosed occasionally
• Unpleasant feelings, interruptions of the heart, palpitation,
periodic cardialgias
• Moderate cardiomegaly mainly due to the LV
• Weakness of heart tones, arrhythmias, functional systolic
murmur, moderate signs of HF (heart failure)
• ECG: arrhythmias, conduction impairments, signs of
hypoxia of myocardium
• Echo-CG: signs of CF, hypokinesia of the left ventricle
CHRONIC CARDITIS
• More frequently is diagnosed as primary-chronic with the
signs of chronic left heart failure;
• Possibly occurs after acute or subacute carditis lasting
more than 12 – 18 months;
• Signs of HF, cardiomegaly, tachy- bradycardia, weakeness
of cardiac tones, cardiac hump;
• Physical retardation, encephalopathy, anaemia;
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ECG: arrhythmias, hypertrophy of the LV
Echo-CG: dilatation of the LV,
decreased retractive function ;
decreased of ejection fraction ;
reduce myocardial contractility.
Congestive Heart Failure
• Decreasing of cardiac output;
• Systolic dysfunction – decreased myocardial
contractility;
• Diastolic dysfunction – insufficient expansion
for ventricular volume;
• Problems are accentuated by increased
metabolic and oxygen demand, developed
severe heart failure
Physical Examination
• Symptoms of heart failure
– pulmonary congestion (left HF)
Dyspnea – breathlessness (rest, exertional, nocturnal),
Orthopnea – dyspnea lying down,
Paroxysmal nocturnal dyspnea – extreme dyspnea,
Bronchospasm.
– systemic congestion (right HF)
edema, enlargement of liver, nausea, abdominal pain, nocturia
– low cardiac output
fatigue and weakness, hypotension, tachycardia, tachypnea
TREATMENT OF CARDITIS
• REGIME: duration of the bed rest is determined according to
the degree of cardiomegaly and cardiac insufficiency, on the
average 2 – 6 weeks with gradual expansion;
• DIET: Table №10, uses of salt and liquid according to the
degree of cardiac failure: excluding piquant products, replacing
them on products with potassium and vitamines;
TREATMENT OF CARDITIS
Acute carditis:
• Antiviral and antibacterial therapy - duration of the antibacterial therapy
should be not less than 3-4 weeks (Penicillin - for elimination the chronic
infectious foci present in most patients with carditis).
• glucocorticoids (prednisolon 0,5-1,5 mg/kg) 2-4 weeks with gradual decreasing
• NSAID - nonsteroid anti-inflammatory drugs (Aspirin 100 mg/kg,
Ibuprophen 10-15mg/kg, Voltaren 2-3 mg/kg, Indometacin 2-3mg/kg Misulid 5-10
mg/kg, Mephenamin acid 50 mg/kg, Amizon 50 mg/kg) - 4 weeks with gradual decreasing
of dose during 2-3 weeks;
End-stage EFE with signs of HF
despite maximal medical treatment,
is an indication for heart transplantation
TREATMENT OF CARDITIS
•Subacute and chronic:
Chinoline derivatives (Delagil 5 mg/kg, Planquenil 8 mg/kg) 4-6 mos,
decrease to 1/2 dose, give by ys
NSAID, Antibiotic therapy is used only at bacterial carditis with high
activity
TREATMENT OF CARDITIS
SYMPTOMATIC THERAPY
Medicines for improvement the function of myocardium:
• Digitalis (Strophanthin 0,012 mg/kg, Digoxin 0,03 - 0,05 mg/kg) at cardiac
insufficiency
•Diuretics (Lasix 1- 3 mg/kg, Spironolactone-Veroshpiron 1-3 mg/kg,
Hypothiazid 2-5 mg/kg);
•ACE inhibitors - peripheral vasodilatations - Captopryl 0,5-1 mg/kg,
•Anticoagulants (Heparin of 100 U/kg), antiaggregants - Curantil 2,5-3mg/kg,
• B2-blocking agents (Metoprolol, Obzidan 1.0 – 2.0 mg/kg)
•Phosphaden, Panangin, Riboxin, Mildronat, Cardonat 1 - 1,5 month
Cardiomyopathies
is an idiopathic abnormality
of myocardial function
There are several
Actuality types of cardiomyopathies defined by the WHO:
“Dilated cardiomyopathy” or DCM,
“Hypertrophic cardiomyopathy” or HCM,
“Restrictive cardiomyopathy” or RCM,
Incidence and Prognosis
• 3-10 cases per 100,000
• 1% of all pediatric cardiac disease
• During infancy incidence is 10 times higher than
older children
• death from progressive pump failure
1-year
2-year
5-year
25%
35-40%
40-80%
• stabilization observed in 20-50% of patient
• Complete recovery is rare
WHO Classification
Primary diseases of the myocardium
Cardiomyopathies
• Primary
(those resulting from genetic
abnormalities of cardiac
muscle)
– Dilated
– Hypertrophic
– Restrictive
• Secondary
(those resulting from infections,
metabolic and nutritional
diseases, endocrine disorders,
neuromuscular diseases,
blood diseases, tumors)
Br Heart J 1980; 44:672-673
Cardiomyopathy
is based on the predominant structural and
functional abnormalities
• Dilated
Cardiomyopathy –
primarily systolic
dysfunction
• Hypertrophic
Cardiomopathy –
primarily diastolic
dysfunction
• Restrictive
Cardiomyopathy primarily diastolic,
but often combined
systolic dysfunction
Idiopathic
Dilated Cardiomyopathy
is characterized by varying degrees of dilatation
of ventricles, most prominently the left.
LV dilatation and systolic dysfunction pathology:
• increased heart size and weight,
• ventricular dilatation, normal wall thickness,
• heart dysfunction out of portion to fibrosis
Dilated Cardiomyopathy
• The cause - a genetic basis or are
the sequelae of viral myocarditis.
• In 20-50 % of cases, the disease is
recognized as familial.
• Autosomal dominant inheritance is
most commonly encountered and
mutations in several cardiac
structural or metabolic genes have
been identified.
Dilated
Cardiomyopathy
• Failure of the LV causes an increase in end-diastolic
volume, which results in increase in LA, pulmonary
venous and pulmonary capillary pressure.
Mitral valve regurgitation may result from papillary
muscle dysfunction or severe dilatation of the valve
annulus.
Clinical Manifestations
• All age group may be affected;
• The onset is insidious, but sometimes symptoms of
heart failure occur suddenly;
• Irritability, anorexia, abdominal pain, cough
from pulmonary congestion and dyspnea with
exertion are common;
• Infants and younger children have respiratory
symptoms and failure to thrive.
Clinical Manifestations
• The skin is cool and pale;
• The arterial pulse is decreased, tachycardia,
palpitation is present.
• Jugular venous pressure is increased, hepatomegaly,
edema are common;
• The heart is enlarged, holosystolic murmurs of mitral
and tricuspid insufficiency may be present;
• Blood pressure may be low, pulse pressure narrow;
• A gallop rhythm is audible.
DIAGNOSIS OF CMP
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•
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Clinical signs
Chest x-ray
CT Scan
Echocardiogram
12-lead ECG
Heart biopsy
MRI (magnetic resonance imaging)
Cardiac catheterization
Dilated cardiomyopathy
Chest x-ray – cardiomegaly, allows evaluation of the degree of
pulmonary congestion and presence of pleural effusions
Cardiac Imaging
• ECG – combination of atrial enlargement,
varying degrees of left or right ventricular
hypertrophy;
EchoCG in patient with DCMP
Echo-CG
• dilatation of the
LA and LV
• poor contractility
Doppler studies
• decreased flow
velocity through the
aortic valve and
mitral regurgitation.
• In long-standing
cases, evidence of
Plm ht may exist.
NON-INVASIVE EVALUATION OF MYOCARDITIS
MRI IMAGING
Enhanced
Friedrich MG et al. Circulation 1998;97:1802-9.
ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY
RIGHT VENTRICULAR
BIOPSY TECHNIQUE
right ventricular
left ventricular
• Serious complications
include ventricular
arrhythmias leading to
syncope and sudden death,
pulmonary or systemic
emboli from intracardiac
thrombi and development of
pulmonary vascular disease
from chronically elevated left
atrial pressure.
Intracardiac thrombi
Prognosis
• The course of disease is progressively downhill,
although some patients may remain stable for
years.
• Treatment of HF may result in temporary
remission, but relapses are common and in
time – patients become resistant to therapy.
• Prognosis for survival beyond a year is poor.
Implantation of cardioverter-defibrillator
Patients with severely
depressed myocardial
function should be monitored
for arrhythmias and, if
present, treated aggressively
with antiarrhythmic agents
or an implantable
cardioverter-defibrillator
(ICD).
Management of DCM
•
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Limit activity based on functional status
salt restriction of a 2-g Na+ (5g NaCl) diet
fluid restriction for significant low Na+
initiate medical therapy
– ACE inhibitors, diuretics
– digoxin, metoprolol, carvedilol
– hydralazine/nitrate combination
Management of DCM
• consider adding ß-blocking agents if
symptoms persists
• Anticoagulation (history of thromboemboli,
presence of mural thrombi)
• intravenous dopamine / dobutamine
• cardiac transplantation
Hypertrophic Cardiomyopathy
idiopathic hypertrophic subaortic stenosis,
idiopathic obstructive cardiomyopathy
The septum is
thickened out of
proportion to the
left ventricular
wall, which may
also be thickened.
Hypertrophic Cardiomyopathy
• Occurrence of 0.02 to 0.2%
• A hypertrophied and non-dilated LV
in the absence of another disease
• Small LV cavity,
• Asymmetrical septal
hypertrophy (ASH),
• Systolic anterior motion of the
mitral valve leaflet (SAM)
Septal
hypertrophy
•Small LV cavity
•Asymmetrical
septal hypertrophy
Hypertrophic
Cardiomyopathy
• Hypertrophy of ventricular
septum (95%)
• Disarray of myofibrils (100%)
• Volume reduction of ventricles
(90%)
• Endocardial thickening of LV
(75%)
• Mitral valve leaflet thickening
(75%)
• Dilated atria (100%)
• Abnormal intramural
coronaries (50%)
The myocardial disarray
•
Microscopic examination of the heart muscle shows that it is
abnormal.
• The normal alignment of muscle cells is absent and this
abnormality is called myocardial disarray.
• This disarray can contribute to an irregular heartbeat
(arrhythmia) in some people.
Pathophysiology
• The main abnormality of the heart in HCM is an excessive thickening of
the muscle. Thickening begins during early adolescence and stops when
growth has finished (to early 20s).
• The hypertrophy is the greatest in
the septum (wall separating the left
and right chambers of the heart).
• The muscle thickening in this
region may be sufficient to narrow
the outflow tract .
Pathophysiology
• In some cases of asymmetric septal hypertrophy, obstruction to the
outflow of blood from the heart may occur, as shown here.
The mitral valve touches the
septum, blocking the
outflow tract.
Some blood is leaking back
through the mitral valve
(mitral regurgitation)
Pathophysiology
65%
35%
• In the thickened, stiff LV systolic
function is well preserved, but
diastolic function is compromised.
• Thickening of the septum may result
in LV outflow obstruction and
abnormal motion of the mitral valve.
• This abnormal motion may result in
mitral regurgitation.
Variants of HCM
Most common location: subaortic, septal, ant. wall
• Asymmetric hypertrophy (septum +
anterior wall) 70 %
• Basal septal hypertrophy: 15- 20 %
• Apical or lateral wall: < 2%
• Concentric LVH: 8-10 %
Clinical Manifestation
Clinical Manifestation
• Asymptomatic, Echo-CG finding
• Symptomatic
– Weakness, fatigue, dyspnea on effort (because of an
inability to significantly increase cardiac output with
exercise) in 90%
– Palpitations, chest pain, angina pectoris (due to
myocardial ischemia) in 75%
– Dizziness, pre-syncope, syncope
 risk of SCD in children and adolescents
Clinical Manifestation
• The pulse can be brisk – early systolic ejection of blood
from ventricle;
• A prominent LV lift, double apical impulse (peaked)
because ejection is interrupted by septal obstruction;
• The 1st and 2nd heart sounds – normal;
• Systolic ejection click
• The systolic murmur is ejection in type and of medium
intensity – is heard maximally at the LSB and apex;
• Murmur may increase shortly after exercise is
discontinued (Valsalva’s maneuver)
Diagnosis
ECG
• LV hypertrophy, ST-segment depression and T-wave
inversion.
• Signs of the WPW (Wolff-Parkinson-White)-syndrome,
• Rhythm disturbances are defined by Holter monitoring.
X-ray chest
• Mild cardiomegaly with prominence of the left ventricle;
Diagnosis
Echo-CG: LV hypertrophy – asymmetric, concentric, apical; systolic anterior motion of
the anterior leaflet of mitral valve
Doppler allow evaluation of mitral valve regurgitation
sept
Patient with
severe
asymmetric LV
hypertrophy;
Sept – septum
LVPW – left
ventricular posterior
wall
LV
LVPW
sept
PROGNOSIS OF HCM
• IS UNPREDICTABLE
• Asymptomatic patient may remain stable
for years.
• Some patients progress to chronic HF,
whereas others are risk for sudden death
from arrhythmia.
Management
• Competitive sports and strenuous physical activity should be
prohibited because most sudden deaths occur during or
immediately after vigorous physical exertion, especially in
adolescents and young adults.
• Digitalis or aggressive diuresis is contraindicated in most
patients because of the potential to increase LV outflow
obstruction
• beta-adrenergic blocking agents (propanolol, atenolol)) and
calcium channel blocking agents (verapamil, nifedipine) may be
useful in diminishing ventricular outflow tract obstruction,
modifying ventricular hypertrophy, and improving ventricular
Management
• Patients with documented arrhythmias or a history of
unexplained syncope should be treated aggressively,
usually with an implantable cardioverter-defibrillator
(ICD).
• Prevention of sudden death with implantable defibrillator is
efficacious
• Surgery treatment
– ventricular septal myotomy (disabling angina, syncope
associated with LV outflow obstruction)
– mitral valve replacement (if obstruction cannot be
alleviated);
DCM v.s. HCM
Restrictive
Cardiomyopathy
Rigid ventricular wall
• Poor ventricular compliance
is major abnormality in
restrictive cardiomyopathies,
and inadequate filling of the
ventricular cavities occurs
during diastole and results in
clinical manifestations
Restrictive Cardiomyopathy
• One of least common cardiomyopathies
• Ventricular volume and wall thickness is
normal
• Decreased volume of both ventricles
• Mostly idiopathic- sometimes familial
• Systemic disorders - amyloidosis, sarcoidosis,
hemochromatosis, scleroderma, and carcinoid.
Restrictive Cardiomyopathies
• Hallmark: abnormal diastolic function
• Rigid ventricular wall with impaired
ventricular filling
• Bear some functional resemblance to
constrictive pericarditis
Treatment
• No satisfactory medical therapy
• Drug therapy must be used with caution
– When signs of heart failure exist, use of diuretics
can result in clinical improvement.
– vasodilators may decrease filling pressure
– ? Calcium channel blockers to improve diastolic
compliance
– digitalis and other inotropic agents are not
indicated!!!
Thank you for attention!